Textbook of Dermatologic Ultrasound 3031087356, 9783031087356

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Textbook of Dermatologic Ultrasound Ximena Wortsman Editor

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Textbook of Dermatologic Ultrasound

Ximena Wortsman Editor

Textbook of Dermatologic Ultrasound

Editor Ximena Wortsman Institute for Diagnostic Imaging and Research of the Skin and Soft Tissues, Santiago, RM, Chile Department of Dermatology, Universidad de Chile, Santiago, RM, Chile Department of Dermatology, Pontificia Universidad Catolica de Chile, Santiago, RM, Chile

ISBN 978-3-031-08735-6    ISBN 978-3-031-08736-3 (eBook) https://doi.org/10.1007/978-3-031-08736-3 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2022, corrected publication 2023 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Foreword

It gives me great pleasure to write the foreword for the Textbook of Dermatologic Ultrasound, edited by Ximena Wortsman, MD. Dr. Wortsman is widely regarded as the world’s expert in this topic, and she has assembled an impressive roster of co-authors. Dermatologic ultrasound is a quickly growing field that has been fueled by vast improvements in ultrasound image quality over the past decade. In a busy imaging practice, we often see dermatologic conditions either as the primary reason for referral or as an incidental finding when scanning for something else. Most practitioners, however, have had insufficient training in the diagnosis and management of these conditions. As a subspecialist in musculoskeletal ultrasound, I often get calls from other radiologists in the department asking my opinion about “lumps and bumps,” but in truth, I have learned everything that I know about dermatologic ultrasound from Dr. Wortsman via her books, lectures, and scientific papers. In fact, I have a copy of her previous book, Dermatologic Ultrasound with Clinical and Histologic Correlations, that I can never keep in my office—it is constantly being borrowed by my colleagues, and the information contained within it has helped countless patients. I expect that this book will be equally popular, and I thank Dr. Wortsman and her co-authors for creating another invaluable educational tool. I’m sure that it will be equally difficult to keep others from borrowing it—but that is fine with me! Levon N. Nazarian, MD, FAIUM, FACR William E. Conrady, MD Professor of Radiology Vice-Chair for Education, Department of Radiology Sidney Kimmel Medical College of Thomas Jefferson University Philadelphia, PA, USA President, American Institute of Ultrasound in Medicine Laurel, MD, USA

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Preface

The Textbook of Dermatologic Ultrasound is a compendium of the most important information that anyone working on dermatologic ultrasound should know. It has 28 chapters that gather the essential knowledge and advances in the field, the experience and views of authors from multiple countries worldwide, and my own expertise. Thus, there is a wide variety of practical topics that include normal anatomy, protocols and settings, common dermatologic pathology, interventional, and tips for reporting the examinations, among many others. The book was written in the middle of the Coronavirus pandemic, allowing us to have some time for the difficult task of collecting and organizing the materials. It includes nice clinical and ultrasonographic correlations with state-of-­ the-art images and some videos, besides histologic photographs. There is also a self-assessment module (appendix) with CME tests that contains 150 multiple-choice questions about the essential concepts of the chapters. I hope this book could support the practice of dermatologic ultrasound professionals around the world and enhance the curiosity of the ones interested or recently approaching this field. Santiago, de Chile November 2022

Ximena Wortsman

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Acknowledgments

I would like to deeply thank all the contributors to this book, particularly Dr. Kharla Pizarro and Dr. Yamile Correidora, both pathologists who allowed us to have the most wonderful histologic correlations and patiently answered my continuous requests. Special thanks to Ms. Geraldine Cocca, Makarena Guerra, Adelina Varela, Veronica Pacheco, and Piroska Kozma, who are members of the staff of the Institute for Diagnostic Imaging and Research of the Skin and Soft Tissues. Last but not least, I would like to thank my parents Gloria and Isaias, my children Benjamin and Camila, and my brothers Claudio and Marcelo, who have always encouraged me to continue growing in this field. My father passed away, but I’ll always be grateful for how he supported my professional development.

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Contents

Part I The Requisites 1 Technical  Recommendations, Settings, Protocols, Training, and Reports of the Dermatologic Ultrasound Examinations����������������������������������������������������������������   3 Ximena Wortsman 2 Training  in Dermatologic Ultrasound��������������������������������������������  17 Fernando Alfageme 3 Dermatologic Concepts and Terminology��������������������������������������  21 Diana Crisan and Maria Crisan 4 Ultrasound  Imaging: Basic Principles and Terminology ������������  73 Diana Gaitini, Yehuda Ullmann, and Marcia Javitt 5 Comprehensive  Ultrasonographic Anatomy of the Normal Skin, Nail, Hair, and Adjacent Structures ����������������������  89 Ximena Wortsman, Camila Ferreira-Wortsman, Yamile Corredoira, and Kharla Pizarro 6 Relevant  Topographic Anatomy of the Head, Anatomical Variants, and Risk Zones�������������������������������������������� 101 Ximena Wortsman and Camila Ferreira-Wortsman Part II Ultrasound Features of Common Dermatologic Conditions 7 Ultrasound  of Congenital Cutaneous Conditions ������������������������ 133 Ximena Wortsman, Kharla Pizarro, Yamile Corredoira, Claudia Morales, and Laura Carreño 8 Nonvascular  Benign Tumors and Pseudotumors�������������������������� 157 Ximena Wortsman, Kharla Pizarro, Yamile Corredoira, Laura Carreño, and Claudia Morales 9 Essential  Concepts on Ultrasonography of Skin Cancer�������������� 185 Ximena Wortsman, Kharla Pizarro, Yamile Corredoira, Laura Carreño, and Claudia Morales

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10 Ultrasound  of Cutaneous Melanoma: Primary Tumor Assessment and Locoregional Staging������������������������������ 213 Orlando Catalano 11 Ultrasound  of Cutaneous Lymphomas������������������������������������������ 251 Anitha Mandava 12 Use  of Ultrasound in Cryosurgical Treatment������������������������������ 259 Paola Pasquali and Myrto-Georgia Trakatelli 13 Basal  Cell Carcinoma Ultrasound Examination with 20 and 75 MHz High-Frequency Ultrasound������������������������ 267 Artur Bezugly 14 Role  of Ultrasound at 50 MHz in Skin Cancer������������������������������ 283 Jie Liu, Yu-Kun Wang, and Qing-Li Zhu 15 Role  of Ultrasound at 70 MHz in Skin Cancer������������������������������ 293 Teresa Oranges, Valentina Dini, and Marco Romanelli 16 Ultrasound of Vascular Tumors������������������������������������������������������ 301 Ximena Wortsman 17 Main  Concepts on Ultrasonography of Dermatologic Inflammatory Conditions���������������������������������������� 315 Ximena Wortsman, Yamile Corredoira, Kharla Pizarro, Laura Carreño, and Claudia Morales 18 Clinical  Overview of Inflammatory Dermatologic Conditions���������������������������������������������������������������� 341 Cristián Vera-Kellet 19 Clinical  Overview of Psoriasis and Psoriatic Arthritis ���������������� 345 Fernando Valenzuela and Rodrigo Flores 20 Ultrasound  of Common Infestations and Infections�������������������� 367 Marcio Bouer and Ximena Wortsman 21 Ultrasound  of Nail Conditions�������������������������������������������������������� 387 Ximena Wortsman, Yamile Corredoira, Kharla Pizarro, Laura Carreño, and Claudia Morales 22 Ultrasound in Aesthetics������������������������������������������������������������������ 415 Ximena Wortsman 23 Cutaneous  Ultrasonography in Pediatric Dermatology �������������� 433 Ana Isabel Rodríguez Bandera Part III Practicalities 24 Tips  for Reporting Dermatologic Ultrasound Examinations������ 487 Ximena Wortsman

Contents

Contents

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25 Ultrasound  Intervention in Tumors, Pseudotumors, and Vascular Soft-Tissue Lesions�������������������������������������������������������������������������� 497 Jose Luis del Cura and Gorka del Cura 26 Ultrasound-Assisted  Hidradenitis Suppurativa Procedures�������� 509 Francisco Javier García Martínez 27 Interventional  Dermatologic Ultrasound in Aesthetics���������������� 517 Fernanda Aquino Cavallieri and Laila Klotz de Almeida Balassiano 28 The  Role of Ultrasound in the Use of Hyaluronidase ������������������ 525 Leonie W. Schelke and Peter J. Velthuis Correction to: Ultrasound in Aesthetics������������������������������������������������� C1 Appendix: Self Assessment Modules (CME Questions) ���������������������� 531 Index���������������������������������������������������������������������������������������������������������� 547

Contributors

Fernando  Alfageme Department of Dermatology, Hospital Universitario Puerta del Hierro Majadahonda, Universidad Autonoma de Madrid, Madrid, Spain Artur Bezugly  Department of Dermatology and Cosmetology, Academy of Postgraduate Education under the Federal State Budgetary Unit Federal Scientific and Clinical Center for Specialized Medical Assistance and Medical Technologies of the Federal Medical Biological Agency, Moscow, Russia Marcio  Bouer Department of Radiology, Fleury Laboratory, São Paulo, Brazil Laura  Carreño Department of Pathology, Dermopathology Section, Universidad de Chile, Santiago, Chile Orlando  Catalano  Department of Radiology, Varelli Diagnostic Institute, Naples, Italy Fernanda Aquino Cavallieri  Clínica Cavallieri, Rio de Janeiro, Brazil Yamile  Corredoira Department of Pathology, Dermopathology Section, Universidad de Chile, Santiago, Chile Department of Pathology, Hospital San Borja Arriaran, Central Campus Faculty of Medicine, Universidad de Chile, Santiago, Chile Diana Crisan  Department of Dermatology and Allergic Diseases, University Clinic Ulm, Ulm, Germany Maria  Crisan Department of Histology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania Dermatology Clinic, Emergency County Hospital, Cluj-Napoca, Romania Laila  Klotz  de Almeida Balassiano Sector of Dermatology and Post Graduation Course in Medical Clinics—HUCFF-UFRJ and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil Gorka  del Cura Department of Radiology, Galdakao-Usansolo Hospital, Galdakao, Spain Jose  Luis  del Cura Department of Radiology, Donostia Unibertsitate Ospitalea, Donostia-San Sebastián, Basque Country, Spain

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Valentina Dini  Department of Dermatology, University of Pisa, Pisa, Italy Camila Ferreira-Wortsman  School of Medicine, Universidad Finis Terrae, Santiago, Chile Rodrigo  Flores Department of Dermatology, Faculty of Medicine, Universidad de Chile, Santiago, Chile Diana  Gaitini Department of Diagnostic Imaging, Rambam Health Care Center and Ruth and Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel Francisco  Javier  García Martínez Dermatology Department, Clínica Universidad de Navarra, Madrid, Spain Marcia  Javitt Department of Diagnostic Imaging, Rambam Health Care Center and Ruth and Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel Jie  Liu Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China Anitha  Mandava Department of Radiodiagnosis, Basavatarakam Indo American Cancer Hospital & Research Institute, Hyderabad, Telangana, India Claudia  Morales Department of Pathology, Dermopathology Section, Universidad de Chile, Santiago, Chile Teresa Oranges  Department of Dermatology, University of Pisa, Pisa, Italy Dermatology Unit, Department of Pediatrics, Meyer Children’s Hospital, Florence, Italy Paola Pasquali  Department of Dermatology, Pius Hospital of Valls, Valls, Tarragona, Spain Universidad de Alcalá Medicine and Medical Specialties, Madrid, Spain Kharla  Pizarro Department of Pathology, Hospital San José, Santiago, Chile Ana  Isabel  Rodríguez Bandera Department of Dermatology, University Hospital La Paz, Madrid, Spain Marco  Romanelli Department of Dermatology, University of Pisa, Pisa, Italy Leonie W. Schelke  Department of Dermatology, Erasmus Medical Centre, Rotterdam, The Netherlands Myrto-Georgia  Trakatelli  Dermatology and Venerology, Second Department of Dermatology, Papageorgiou Hospital, Aristotle University School of Medicine, Thessaloniki, Greece

Contributors

Contributors

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Yehuda Ullmann  Plastic and Aesthetic Department, Rambam Health Care Center and Ruth and Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel Fernando  Valenzuela Department of Dermatology, Faculty of Medicine, Universidad de Chile, Santiago, Chile Department of Dermatology, Clinica Universidad de los Andes, Santiago, Chile Peter  J.  Velthuis Department of Dermatology, Erasmus Medical Centre, Rotterdam, The Netherlands Cristián  Vera-Kellet Department of Dermatology, School of Medicine, Pontificia Universidad Catolica de Chile, Santiago, Chile Yu-Kun  Wang Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China Ximena  Wortsman  Institute for Diagnostic Imaging and Research of the Skin and Soft Tissues, Santiago, RM, Chile Department of Dermatology, Universidad de Chile, Santiago, RM, Chile Department of Dermatology, Pontificia Universidad Catolica de Chile, Santiago, RM, Chile Qing-Li  Zhu Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

Part I The Requisites

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Technical Recommendations, Settings, Protocols, Training, and Reports of the Dermatologic Ultrasound Examinations Ximena Wortsman

Introduction

actual number of corporal regions affected by hidradenitis suppurativa or assessing the degree Due to the development of high- and ultrahigh-­ of regression of an infantile hemangioma [1, 7, frequency probes and more sensitive machines, 13, 14]. dermatologic ultrasound has dramatically Thus, ultrasound is an essential diagnostic expanded in the last decade [1–3]. Moreover, tool in the arsenal of dermatology that provides ultrasound has followed a validation process that data that is impossible to have with other imaging includes the reports of the accuracy of the tech- techniques such as MRI or CT, and even with nique, the description of the normal patterns, the dermoscopy, confocal microscopy, or optical standardization of the image acquisition and coherence tomography [3, 7, 14]. reports, the publications of guidelines, the use of In this chapter, we review the current recomadequate and updated machines with trained mendations, settings, protocols, training, and operators, the qualification and quantification of reports of these exams. abnormalities, and the description of the limitations and a multicentric reproducibility [1–12]. These processes have taken a long but necessary Recommendations and Relevant time to assess quality standards. Points Nowadays, this type of examination is practiced in multiple countries and solves several There are essential requisites for performing the daily dermatologic critical issues. Moreover, this dermatologic ultrasound examinations [4, 12], information cannot be deducted from the clinical which are: examination or the histologic exam. Among the multiple examples of this fact are knowing the 1. A color Doppler ultrasound machine working with a linear or compact linear multifrequency probewith a maximum  frequency  range ≥15 MHz. X. Wortsman (*) 2 . A trained operator in dermatologic conditions Institute for Diagnostic Imaging and Research of the and ultrasound imaging. Skin and Soft Tissues, Santiago, RM, Chile Department of Dermatology, Universidad de Chile, Santiago, RM, Chile Department of Dermatology, Pontificia Universidad Catolica de Chile, Santiago, RM, Chile

So far in the market, there are multiple machines with that capabilities. However, testing the dermis’ definition and the blood flow’s sensitivity

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 X. Wortsman (ed.), Textbook of Dermatologic Ultrasound, https://doi.org/10.1007/978-3-031-08736-3_1

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X. Wortsman

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before buying is mandatory. Currently, most brands do not provide a dermatologic ultrasound configuration for their machines. Therefore, it is necessary to call the company’s application ­specialist and work on the dermatologic examinations’ best settings. Regarding the frequency, there are several options as well. It should be kept in mind that at higher frequencies, the penetration is lower [8]; therefore, it may be necessary to have at least two probes with different ranges of frequencies that could give you a high definition of the cutaneous and deep layers. Nowadays, the market can provide color Doppler machines with multifrequency probes that go up to 70 MHz [7–9, 15]. The axial spatial resolution of ultrasound is much higher than MRI devices. For example, a 15 MHz probe’s axial resolution is 100 μm and a 70 MHz probe is 30 μm [15]. In contrast, at 3.0 T, MRI devices present an axial resolution of 400 μm, and when working at 7.0 T a resolution of 100 μm [15]. This is critical for studying the most superficial structures, which benefit from the better resolution of ultrasound than MRI or CT. In practical terms, it would be impossible to detect tiny structures such as a hair follicle on the usual commercial devices of MRI or CT because, to date, they present a lower axial spatial resolution.

 ow to Improve the Learning of H Dermatologic Ultrasound Coordination of the work considering dermatology, imaging (radiology), and pathology is encouraged [1, 4, 5, 7–9, 12, 14, 16, 17]. This allows us to have a proper feedback on the interpretation of the images. There are many dermatology clinical books, and during the past decade, several dermatologic ultrasound books have been published [8, 9]. Additionally, there are courses on dermatologic ultrasound in well-known international scientific societies such as the AIUM (American Institute of Ultrasound in Medicine; www.aium.org) and the EFSUMB (European Federation of Societies of Ultrasound in Medicine and Biology; www.efsumb.org), among others.

Protocols for Performing Dermatologic Ultrasound Examinations This examination comprises a sequence that includes the patient’s clinical examination, the performance of a grayscale and color Doppler examination, and the lesional vessels’ spectral curve analysis, also called pulsed Doppler (Fig. 1.1) [4, 5, 9, 12].

Fig. 1.1  Protocol for performing a dermatologic ultrasound examination

1  Technical Recommendations, Settings, Protocols, Training, and Reports of the Dermatologic…

The patients’ clinical examination includes the palpation of the lesion(s) and extracting the relevant parts of patients’ history. For adequate performance, the room should be well illuminated so that it can be possible to dim the lights during the ultrasound examination to improve the screen’s view [8, 9, 12]. Certain types of examinations require extending the field of study, and bilateral or multiple regions should be revised on ultrasound. One reason to back up these extended studies is that comparative examination facilitates information capture. Besides, there is a need to track subclinical activity in inflammatory conditions [18, 19], and it is necessary to detect the involvement of additional structures in aesthetic patients [20]. Examples of these bilateral or multiple regions’ studies are: 1. Nail studies. 2. The staging and tracking of activity in hidradenitis suppurativa. 3. The tracking of subclinical activity in morphea. 4. The detection of subclinical inflammatory signs in the facial glands of users of cosmetic fillers. 5. The locoregional staging of skin cancer. The objective is to perform an anatomic and functional examination that could capture a high amount of critical data that could potentially change the patient’s management. Moroever, a proper planning of the examination time is of paramount importance for the adequate performance and interpretation of the examination. [1– 5, 7–9, 11, 12, 14, 17].

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Sedation can also be avoided in newborns or children ≤3  months under lactation. Hence, these children could be fed by their mothers during the examination [7–9]. We use chloral hydrate for sedation, 50  mg/ kg, administered orally 30 min before the examination. A second dose could be given if the child does not fall to sleep in 30 min. If the child is still awake with the second dose, we reschedule the examination with a strict rule of sleep deprivation and match the child’s nap hour. It is essential to instruct the parents or guardians not to bring the child to the examination sleeping in the car or after a nap. The collaboration of the parents or guardians is critical for achieving a comfortable and helpful examination [7–9]. Signed informed consent is necessary to administer chloral hydrate; therefore, the child’s appointment is usually scheduled 1 h before the examination. Hence, the parents can have enough time to read and sign the consent and ask questions [7–9]. During the examination, the child is monitored with a pulse oximeter and following the modified ALDRETE score (consciousness, activity, respiration, circulation (blood pressure), and oxygen saturation) and only discharged when the patient is awake (Fig. 1.2) [21]. In our experience, the effect of the single dose of chloral hydrate lasts approximately 20–40 min, and the double dose lasts for 1–2  h. Of course, this could have variability. For sedation cases, it is crucial to have a separate room from the general waiting room so that the children can be monitored easily and privately.

Sedation The performance of sedation is recommended in children ≤4 years. This is because the child’s crying or movement generates noise on the screen, and it is difficult to capture the data in a standardized way. Of course, the operator should be flexible and evaluate case to case because children could be quiet despite their age or may be helped with the use of smartphones and multimedia.

Fig. 1.2  Sedation should be monitored with a pulse oximeter

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Alternatives to sedation, such as cartoons or movie clips available in interactive media on tablets or cell phones, can easily distract older children and improve the examination’s performance. However, in any situation, the collaboration of the parents or guardians is essential.

X. Wortsman

may be used in these cases.  The gel should be inserted within and at the surface of these covers to provide a proper acoustic transmission.

Technique for the Dermatologic Ultrasound Examination After a conversation with the patient and the lesion’s clinical examination, we apply a copious amount of gel on top of the skin or nail (Fig. 1.3). To avoid the hand’s flotation in the gel, we stabilize the hand with the fifth finger’s extension and support (Fig.  1.4). A significant amount of gel can help us with sound transmission and better adjust the screen’s focal zone. We do not use standoff pads because they may compress the skin’s tiny vessels and are impractical when studying large corporal regions [3, 7–9, 12, 14]. However, this is just a recommendation, and their use in dermatologic examinations is not forbidden.  Sterile gel, commercially available, ­ can be used in interventional dermatologic ultrasound procedures. A protective cover of the probe such as for example a condom or a plastic sleeve

Fig. 1.4  To avoid the hand’s flotation in the gel, you can stabilize the hand with the fifth finger

Fig. 1.3  The difference in the amount of gel needed for performing the dermatologic ultrasound between soft-tissue examinations

1  Technical Recommendations, Settings, Protocols, Training, and Reports of the Dermatologic…

The technique is to perform a slow sweep in at least two perpendicular axes. First, we use grayscale and then color Doppler. Power Doppler for finding slow flow vascularity is recommended in devices where the color Doppler is not so sensitive. Additionally, you can use echoangio software for detecting thin vessels. This software subtracts the tissue and leaves only the vessels on the screen, which is similar to angiography. This can be useful for studying vascular lesions because this clearly shows the lumen of the vessels [7–9, 14]. In our experience, elastography has presented an erratic performance on cutaneous and ungual lesions with many false positives and negatives, probably due to the low sensitivity for discriminating stiffness on higher frequencies. The development of more sensitive elastography tools is necessary because this sounds logical for studying many dermatologic conditions and improving “benign versus malignant” or “fibrotic vs. non-­ fibrotic” entities’ discrimination [22]. In our center these days, we use two devices, one that goes up to 24 MHz and the second that goes up to 70 MHz. The examination always starts with the machine that works in the range between 15 and 24  MHz, and then we look at the lesion with the other machine that goes up to 70 MHz, which is particularly helpful in lesions located in the epidermis and dermis. These ultrahigh-­ frequency machines working at 70 MHz are currently available in a few institutions worldwide, usually academic and research centers; however, this may change in the future. Thus, what is mandatory for performing a dermatologic ultrasound examination is to start with a frequency ≥15 MHz. This will give you critical information on the patterns and vascularity of the lesions. The third part of the protocol is the spectral curve analysis of the blood flow. This is relevant to discriminating the arterial and venous flow as well as the presence of arteriovenous shunts. It should be kept in mind that the cutaneous and ungual vessels present low velocity (≤15 cm/s); therefore, it is not likely to track the carotid artery. The spectral curve analysis’s vascular patterns may contribute to a vascular lesion or a tumor’s characterization and track the degree of inflammatory disease activity. We capture the

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arterial vessels’ peak systolic velocity and make at least three samples for nonvascular and six samples for vascular lesions. Then, we report the maximum peak systolic of the vessels in cases with arterial flow [9]. For academic purposes, we classify the maximum peak systolic velocity of the vessels into [9]: Low velocity: ≤15 cm/s. Medium velocity: 15–35 cm/s. High velocity: ≥36 cm/s. Normal skin and nails usually show low-­ velocity flow. With the commonly used machines, it is usually not possible to detect color Doppler signals in the normal dermis due to the threshold of detection of the machines, which is commonly 2  cm/s. However, in some cases and corporal locations, one or two thin isolated vessels may be detected in the dermis and are considered normal. For simplicity and academic purposes, in the descriptions of the images in this book we will use the highest frequency of the probes (greyscale).

Training of Dermatologic Ultrasound Main Concepts There are several training types, and among them, we can mention the operator’s self-­learning by studying the literature and following their cases. The second is Web learning, which could be done through internet courses. So far, there is a growing number of these internet courses, some of them under the umbrella of scientific organizations such as the AIUM or the EFSUMB. Of course, some live lectures and courses are inserted in congresses or meetings of variable sizes. The ones that present a mix of theory and practice are among the most useful; however, these courses are challenging to organize to the usual privacy restrictions of international scientific societies’ big meetings that do not allow patients inside the sessions. In a publication of the DERMUS group, a minimum of a two-day training course with a mix

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of lectures and workshops was recommended [11].This mentions a three-level program that could allow the progressive learning of this technique in steps, validated by a test at the end of the activities.

Assessment of Competence and Operators in Charge of the Examination This could be a controversial topic, but according to the guidelines for performing dermatologic ultrasound examinations, the minimum number for assessing competence is 300 examinations per year. This amount of examination is more or less similar to the number requested for other types of ultrasound examinations, according to the AIUM [12]. In countries with no accreditation of the practices, assessing these quality standards may be challenging to prove, so that the ultrasound quality will rely on the operator’s expertise. Another controversial topic is the designation of the operator in charge of the examination and interpretation. According to the guidelines for performing dermatologic ultrasound examinations, the operator in charge should be a physician. This is because dermatologic ultrasound examinations gather clinical and ultrasonographic information that could jointly support the diagnosis [12].

 ajor Advantages of M the Dermatologic Ultrasound Examinations These advantages include the following [1–5, 8, 12, 14, 17]: 1. The examination’s real-time capability and safety (non-radiating technique) allow detection of the lesions with their vascularity at every stage of life, including in children and pregnant women. 2. The high definition of the examinations can show a wide range of alterations in superficial and deep layers.

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3. The lack of significant problems with the penetration: This is a big advantage of ultrasound over other imaging techniques used in dermatology, such as dermoscopy, confocal microscopy, and optical coherence tomography (OCT). In the latter techniques, there is no chance to have a broad view of the epidermis, dermis, and hypodermis because the penetration varies across a maximum of 200–250 μm in dermoscopy and 1.5–2.0 mm in OCT. Particularly in OCT, the bottom part of the images turns black or blurry beyond the level of light penetration, which can be critical in assessing a skin tumor’s real depth. 4. The capability to show a wide range of alterations: Ultrasound allows the study of several corporal regions in the same patient, which has a decisive advantage over pathology, which can get only a tiny sample of the tissues. This is important for supporting the diagnosis, staging of severity, and tracking activity in inflammatory diseases. Another point is that ultrasound can show the whole lesion, and pathology only a part of the lesion. 5. There is no radiation because this technique uses sound waves. This is an essential difference in comparison with CT. 6. There is no exposure to magnetic fields, allowing patients with pacemakers and metallic prostheses to undergo examinations. 7. There is no need to inject a contrast medium, which is a relevant difference compared to MRI and CT. The contrast medium presents potential adverse reactions such as cutaneous nephrogenic fibrosis and kidney disease. 8. The high axial spatial resolution makes ultrasound a powerful tool compared to MRI and CT. 9. Ultrasound has easy access to all the body surfaces regardless of the shape or contour. For example, it can detect lesions within the concavities or convexities of the face or ear pinna. 10. The interactive ultrasound modality allows a rich clinical information interchange and dynamic maneuvers’ performance.

1  Technical Recommendations, Settings, Protocols, Training, and Reports of the Dermatologic…

Major Limitations Among the current limitations are the following [3, 7–9, 15]: 1. The detection of pigments, such as melanin: This limitation may be perhaps solved with the advances in the development of photoacoustics. 2. The threshold for detecting lesions working on 15–18 MHz is 0.1 mm and on 70 MHz is 0.03 mm. So, when the lesions present higher diameters than those, they can have a better chance of being detected. 3. There are some issues with detecting only epidermal lesions; however, these problems are lower when ultrahigh-frequency probes (50– 70 MHz) are used. 4. In some cases, the hyperkeratosis could show a potent posterior acoustic shadowing artifact, hiding an underlying lesion. A good tip to overcome this is to wait for 3–5  min to increase the tissues’ humidity after a copious application of gel and improve the transmission of sound waves. Another option is to look from the lateral borders making a coronal sweep of the lesions.

Main Applications The main dermatologic ultrasound applications include skin tumors and pseudotumors, nail conditions, vascular lesions, inflammatory dermatologic diseases, and aesthetics [2–4, 7–9, 12, 14]. The number of entities in each category has been increasing over the last decade, and so far, there are some mandatory applications of ultrasound in dermatology. Among these critical requests are assessing the extent, including the depth of a skin or nail tumor, tracking the activity in morphea, assessing the severity and tracking the activity in hidradenitis suppurativa, and discrimination of cosmetic fillers [2–4, 7–9, 12, 14, 19, 20, 23].

Reporting the Examinations The reports of the dermatologic ultrasound examination should contain critical information such as:

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1. Origin of the lesion (dermatologic versus non-dermatologic; endogenous versus exogenous). 2. Exact anatomical location of the lesion, including the corporal regions and layers involved. 3. Extent in all axes (cm or mm). 4. Shape (oval, round, saclike, band-like). 5. Echogenicity patterns (anechoic, hypoechoic, hyperechoic). 6. Definition of the borders (well or ill defined). 7. Relevant artifacts that support the diagnosis (posterior acoustic shadowing or reinforcement, mini-comet tail artifact, diffuse reverberation, etc.) 8. Adjacent vital structures such as glands, muscles, or vessels. 9. The phase of the lesions (proliferative, partial, or total regression in infantile hemangiomas; active versus inactive in ­ inflammatory conditions). 10. Sonographic scoring such as SOS-HS, sonographic scoring of hidradenitis suppurativa or SOS-acne, sonographic scoring of acne. 11. Patterns of vascularity (hypovascular, hypervascular, type of flow, velocity of flow cm/sec). 12. Assessment of benignancy or malignancy. 13. Final impression with a presumptive or some differential diagnoses if the lesion is not so typical (no more than three differential diagnoses). Although radiologists tend to perform structured and short reports of the examinations, the reports may be longer than usual in the dermatologic field. This has been requested by ­dermatologists who prefer detailed information instead of just a couple of lines with a conclusion. Delivering inconclusive reports such as an indeterminate nodule or suggesting sending the patient to MRI or histology are discouraged. Indeed, most of these inconclusive reports are generated by problems related to the devices or the operator training. Besides, as we know, MRI does not have a higher axial spatial resolution than ultrasound [15], and the decision to perform a biopsy will depend on the clinician. For sure, an inconclusive report is not useful to anyone.

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 ommon Issues in Dermatologic C Ultrasound There are issues derived from the lack of proper equipment or the operator’s training. These include examinations performed with lower frequency probes (1 cm), elevated, palpable, circumscribed solid skin lesion; can involve all skin layers and can vary in consistency (firm, soft, rubbery, hard, etc.); a large nodule is referred to as a tumor Clinical disorders: Neurofibroma, skin metastasis, cysts, basal cell carcinoma, squamous cell carcinoma Vesicle

Herpes zoster

Definition: Small (5 mm), elevated, circumscribed, fluid-containing (clear, serous, hemorrhagic) cavity; can evolve towards erosions or ulcerations Clinical disorders: Bullous dermatosis (bullous pemphigoid, linear IgA dermatosis), burn or suction blisters, fixed drug eruption Pustule

Folliculitis

Definition: Small, elevated, circumscribed lesion containing purulent exudate (neutrophils), which can be infectious or sterile; may arise in the hair follicle or independently Clinical disorders: Acne vulgaris, rosacea, folliculitis, pustular psoriasis, acute generalized exanthematous pustulosis Wheal/hives

Urticaria

Definition: Irregular, palpable, transient elevated edematous skin lesion due to edema of the papillary dermis, which often changes in size and shape (round, gyrate, irregular, etc.) Clinical disorders: Urticaria, urticaria vasculitis Photographs courtesy: Clinic of Dermatology and Allergology, University Clinic Ulm, Germany

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3  Dermatologic Concepts and Terminology

Table 3.2  Secondary dermatologic lesions—lesions that result from the evolution of primary lesions or by scratching, irritating or secondary infection [1–4] Type of lesion Crust

Definition

Herpes simplex

Definition: Dried exudate (serum, blood, or purulent material) on the surface of a primary lesion Clinical disorders: Impetigo contagiosum, healing phase of herpes Scale

Atopic eczema

Definition: Hyperkeratotic material (increased accumulation of superficial epidermal cells) of different morphology (large, pityriasiform, adherent, loose) Clinical disorders: Psoriasis, eczema, tinea, ichthyosis Fissure

Cheilitis

Definition: linear, painful cleft/split of the epidermis and dermis with sharply defined walls; consequence of marked skin thickening and drying Clinical disorders: Cheilitis, chronic hand eczema (continued)

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28 Table 3.2 (continued) Type of lesion Erosion

Definition

Pemphigus vulgaris

Definition: Superficial, focal loss of the epidermis, usually as a result of intraepidermal or subepidermal cleavage of vesicles or bullae; moist, at later stages crusted lesion which heals without scarring Clinical disorders: Bullous dermatoses (pemphigus vulgaris), traumatic burns, Stevens-­ Johnson syndrome Ulceration

Ectyma

Definition: Deeper defect involving the epidermis, dermis, and sometimes subcutis, which heals with scarring; different sizes, shapes, depths depending on underlying pathology may have elevated, undermined, hard borders Clinical disorders: Venous, arterial, neuropathic ulcers, ecthyma, pyoderma gangrenosum, decubitus Excoriation

Atopic eczema

Definition: Superficial, exogen-­induced, often linear skin erosion, mainly caused by scratching Clinical disorders: Prurigo, atopic dermatitis, arthropod bites

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Table 3.2 (continued) Type of lesion Atrophy

Definition

Morphea

Definition: Thinning of the epidermis and/or dermis leading to shining, often depressed lesions with loss of skin texture Clinical disorders: Lichen sclerosus, anetoderma, striae, morphea Lichenification

Atopic eczema

Definition: Increased epidermal thickening with accentuation of natural skin lines secondary to chronic inflammation and scratching Clinical disorders: Lichen simplex chronicus, atopic dermatitis, pruriginous disorders Scar

Acne scars

Definition: Abnormal formation of connective tissue following local injury or surgery, implying dermal damage; fresh scars appear initially thick and pink, becoming white and atrophic in time Clinical disorders: Postsurgical scarring, acne scars Photographs courtesy: Clinic of Dermatology and Allergology, University Clinic Ulm, Germany

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30 Table 3.3  Special skin lesions [1] Cyst

Epidermal cyst

Definition: Circumscribed lesion with a wall and a lumen containing fluid or solid matter Clinical disorders: Pilar cyst, epidermal cyst Telangiectasia

Rosacea

Definition: Dilated superficial blood vessels Clinical disorders: Rosacea, spider angioma Petechiae

Vasculitis

Definition: Circumscribed blood deposit 5 mm in diameter Clinical disorders: Vasculitis, senile purpura, purpura fulminans Photographs courtesy: Clinic of Dermatology and Allergology, University Clinic Ulm, Germany

Table 3.4  Palpatory features of cutaneous lesions [1, 3] Hard (e.g., Soft (e.g., intradermal nevus, skin tags) Firm (e.g., clavus, callus, calcinosis cutis) dermatofibroma, sebaceous hyperplasia) Compressibility Compressible (e.g., venous lake, Non-compressible (e.g., fibrous papule, basal cell neurofibroma) carcinoma) Tenderness Tender (e.g., angiolipoma, leiomyoma, Non-tender (e.g., sebaceous hyperplasia) glomus tumor, erythema nodosum) Blanching Blanchable (e.g., erythema, urticaria) Non-blanchable (e.g., purpura, vasculitis) degree Texture changes Rough (e.g., actinic keratosis, chronic Smooth (e.g., infiltrated basal cell carcinoma, sweet eczema, psoriasis) syndrome) Fixation Mobile (e.g., lipoma) Fixed to underlying structures (e.g., skin neoplasms, dermatofibroma) Skin Normal temperature (e.g., skin Elevated temperature (e.g., inflammatory disorders, temperature neoplasms, eczema) erysipelas) Pulsatility Pulsatile (e.g., angiosarcoma) Non-pulsatile (e.g., solid tumors) degree

Consistency

Besides recognizing the type of skin lesions, it is also important for the clinicians to have a look at their distribution pattern on the skin, whether the lesions appear in a particular body area (e.g., palms, mucosal membranes, scalp), have a symmetric or asymmetric distribution, or appear in relation to sun-exposed or sun-protected areas. Several skin disorders present, for instance, specific distribution patterns which can easily guide the diagnosis: psoriasis usually involves the scalp, extensor surfaces of elbows and knees, umbilical

area, and gluteal cleft; lichen planus is usually seen on the wrists, forearms, lower extremities, and genital area; herpes zoster usually appears in a dermatomal segment; lupus erythematosus mainly involves sun-exposed areas of the face, whereas other inflammatory conditions such as hidradenitis suppurativa are seen in intertriginous areas such as the axilla, groin, and submammary region. An overview of the most important distribution patterns, which help clinicians in orienting their clinical diagnosis, is provided in Fig. 3.3.

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Symmetric vs. asymmetric distribution Extensor vs flexural surface Unilateral vs.

(skin folds), palms and soles, acral localisation, affecting

bilateral

distal extremities (ears, fingers,

distribution

toes, nose etc)

Distribution patterns Photoprotected Follicular vs.

vs.

perifollicular lesions

photoexposed area

Linear, grouped (herpetiform), Generalized vs.

random, along Blaschko lines,

localized eruption

along cleveage lines, pressure areas, seborrheic areas etc.

Fig. 3.3  Common distribution patterns of cutaneous lesions [5]

Dermatopathological Terms Cutaneous sonography is a true biomicroscope that provides real-time images comparable to histological pictures. Knowledge of cutaneous histology and the main histopathological processes

that define a particular lesion represents valuable support for dermatologists and sonographers. Table 3.5 includes the definitions of the main histological terms used in the diagnosis of dermatological diseases (Table 3.5).

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Table 3.5  Basic dermatopathological terminology [6–8] Acanthosis Acantholysis

Atrophy Ballooning degeneration Corps ronds Civatte bodies Collagen entrapment Cornoid lamellae Epidermotropism Exocytosis Flame figure Follicular mucinosis Granulomas Henderson-Paterson body Hypergranulosis Hyperkeratosis Interface dermatitis

Increase in thickness of the epidermis with elongation of the rete ridges (e.g., psoriasis, chronic eczema) Loss of cell-to-cell adhesion between keratinocytes within the epidermis, inducing the formation of intratissular cavities or bullae (e.g., pemphigus vulgaris, impetigo, epidermolysis bullosa) Reduction in thickness of the epidermis (e.g., lichen sclerosus), dermis (e.g., atrophoderma), or adipose tissue (e.g., deep morphea) Destruction of the epidermis due to dissolution of cell attachments and intracellular edema (e.g., herpes simplex) Rounded nucleus with a halo of pale-pink dyskeratotic cytoplasm (e.g., Darier disease) Pink, globular remnants of keratinocytes (e.g., lichen planus) Collagen ball consisting of collagen fibers surrounded by histiocytes (e.g., dermatofibroma) Parakeratotic skin scale above a localized area of hypogranulosis and dyskeratotic keratinocytes (e.g., porokeratosis Mibelli) Presence of lymphocytes in the epidermis in the absence of spongiosis (e.g., mycosis fungoides) Presence of lymphocytes in the epidermis associated with spongiosis (e.g., spongiotic dermatitis) Collagen incrusted with granules of major basic proteins from eosinophils (e.g., Wells’ syndrome) Destruction of hair sheath anatomy due to deposits of mucin (e.g., alopecia mucinosis) Aggregates of histiocytes with or without multinucleate giant cells (e.g., tuberculosis, granuloma annulare, sarcoidosis, foreign-body granuloma) Intracytoplasmic pink inclusions of molluscum contagiosum

Thickening of the granular layer (e.g., lichen planus) Increased stratum corneum (e.g., psoriasis) Lichenoid-like reaction at the interface of the epidermis and dermis with lymphocytic infiltrate along the dermo-epidermal junction, necrosis of keratinocytes, and vacuolization of the basement membrane area (e.g., lupus erythematosus) Leukocytoclasia Disintegration of neutrophilic nuclei into fragments or nuclear dust (e.g., vasculitis) Munro’s microabscess Clusters of neutrophils in the stratum corneum (e.g., psoriasis) Orthokeratosis Stratum corneum without nuclei (normal epidermis) Parakeratosis Stratum corneum with retained nuclear material (e.g., psoriasis) Palisading Picket-fence-like peripheral arrangement of the cells (e.g., basal cell carcinoma) Pseudoepitheliomatous Acanthosis of the epidermis and adnexal epithelium mimicking a squamous cell carcinoma hyperplasia Schaumann body Laminated calcified structure (e.g., sarcoidosis) Spongiform pustule of Neutrophils in the stratum spinosum, associated with peripheral spongiosis (e.g., psoriasis) Kogoj Spongiosis Intercellular edema of the epidermis (e.g., atopic dermatitis, contact dermatitis)

 lossary of Common Dermatologic G Disorders Dermatologic conditions can be divided into several categories (inflammatory, neoplastic, inherited, etc.) depending on their type. Inflammatory skin disorders can be of either infectious (bacterial, viral, fungal, protozoal) or noninfectious

e­ tiology (autoimmune bullous or connective tissue disorders, urticaria, papulosquamous dermatoses). Neoplastic skin lesions can be categorized as either benign or malignant. At the same time, other categories involve skin diseases arising due to genetic, metabolic, toxic, or traumatic events and developmental abnormalities. The following glossary contains a list of common dermatologic

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conditions and terms that sonographers might encounter when dealing with dermatologic patients [1, 3, 4, 9].

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A –– Abscess: a localized collection of pus developing in the tissue in a non-preformed cavity due to an acute bacterial infection, which usually presents as a hot, red, swollen, and painful nodule; risk factors for abscess development are bacterial overgrowth, immunosuppression, trauma, etc. –– Acanthosis nigricans: the presence of asymptomatic, symmetrical gray-brown, verrucous plaques of the major flexures (neck, groin, axillae) commonly present in obese subjects with insulin resistance, paraneoplastic subjects in the presence of internal malignancy (e.g., gastric cancer), or subjects taking certain medications (contraceptives, glucocorticoids, nicotinic acid, etc.). –– Accessory tragus: benign congenital anomaly of the first branchial arch, presenting mainly in the preauricular area as a small skin-­ colored nodule anterior to the tragus, consisting of skin, subcutaneous fat, and cartilage and covered by vellus hairs; it can appear as a single lesion or in association with preauricular pits, hearing impairment, or malformation syndromes. –– Acrochordon: singular or multiple, benign, skin-colored, pedunculated papules commonly located on the neck or axilla (also known as skin tags) most common in obese subjects and women; possible associations with pregnancy and acanthosis nigricans; asymptomatic unless irritated by jewelry, friction, etc. –– Acne keloidalis nuchae: idiopathic, chronic inflammatory, follicular, papular-pustular eruption of the nape of the neck, and occipital scalp region, commonly seen in males of African descent; the condition leads in time to the development of keloid-like lesions, subcu-

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taneous abscesses, and scarring alopecia in affected areas. Acne vulgaris: chronic, inflammatory disorder of the pilosebaceous unit, leading to obstruction of the pilosebaceous canal, resulting in  local inflammation and formation of comedones, papules, pustules, nodules, and, in severe cases, scarring; acne conglobata refers to a severe form affecting not only the face but also the chest and back of the patients, presenting with multiple inflamed nodules, pseudocysts, and scarring. Acrokeratosis verruciformis: autosomal dominant genodermatosis, sometimes associated with Darier disease, presenting with multiple, benign, asymptomatic, symmetrical keratotic papules resembling plane warts on the dorsum of hands and feet. Actinic keratosis: common precancerous skin lesion occurring in sun-damaged areas such as the face, neck, and dorsal aspects of the hands, usually presenting as single or multiple erythematous, rough, scaly patches/ plaques in middle-aged and elderly subjects; the surrounding skin usually also presents signs of actinic damage (atrophy, pigmentary changes, telangiectasia); in case of uncertainty, or in elevated, hyperkeratotic lesions, a skin biopsy can rule out the progression towards a squamous cell carcinoma. Actinic prurigo: pruritic skin disorder due to increased photosensitivity in children, presenting with small, erythematous, intensely itchy papules, thickened plaques, and nodules in sun-exposed areas, which appear hours or days after sun exposure; lesions often persist throughout childhood and tend to fade in adolescence. Actinic purpura: common benign disorder resulting as a consequence of sun-induced damage to the dermal connective tissue and extravasation of blood into the dermis; actinic purpura presents as violaceous patches (ecchymosis) on the extensor surfaces of the forearms and dorsum of hands often in elderly individuals.

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–– Actinomycosis: a chronic, granulomatous hair loss without inflammation, usually bacterial infection caused by Actinomyces 3–4  months after a precipitating event (psyisraelii, presenting with the formation of an chologic stress, postpartum period, crash abscess and draining sinus tracts, which drain diets, major surgery) or medication (beta-­ purulent material, mainly in subjects with blockers, contraceptives); scarring alopecia poor hygiene, immunosuppressive status, and refers to permanent hair loss due to the malnutrition or in tropical areas; the cervicodestruction of the hair follicle, as seen in lupus facial actinomycosis is the most common erythematosus, lichen planopilaris, frontal type and presents with a slowly progressive, fibrosing alopecia, folliculitis decalvans, acne non-tender, hard nodule of the cheek or the keloidalis nuchae, etc. [11]. jaw, which evolves into an abscess with drain- –– Anetoderma: focal loss of dermal elastic tising sinus tracts. sue, leading to the appearance of localized –– Acute generalized exanthematous pustuloskin depressions, which can appear primarily sis (AGEP): rare pustular drug eruption, or secondarily to unrelated skin conditions, mainly after intake of certain drugs (beta-­ such as infections (chicken pox, syphilis), lactam antibiotics, macrolides, antimalarials, tumors (juvenile xanthogranuloma, pilomacalcium channel blockers), which usually trixoma), inflammatory skin conditions (acne, emerges within a few days of exposure to the granuloma annulare), or systemic diseases culprit drug; clinically, patients present with (Lyme disease, lupus). rapidly spreading erythematous areas on the –– Angioedema: mucocutaneous swelling of the face, armpits, and groin, with multiple sterile dermis and subcutaneous or submucosal tispustules, which can spread to the rest of integsue due to mast cell activation and degranulaument as well as with accompanying fever and tion with release of vasoactive mediators malaise; eventually, as the condition resolves, (histamine, bradykinins, etc.), which can the skin desquamates. involve the tongue, lips, and larynx and lead to –– Adiposis dolorosa (Dercum disease): a airway obstruction (medical emergency); rare condition characterized by the appearangioedema can have multiple causes: herediance of multiple painful lipomas everytary (deficiency of C1 esterase inhibitor), where on the body; commonly associated infectious (herpes simplex, coxsackievirus, with obesity, asthenia, fatigue, and mental streptococcus, etc.), insect bites, physical facdisturbances [10]. tors (cold, pressure), drugs (ACE inhibitors), –– Alopecia: localized or diffuse hair loss, idiopathic, etc. involving the scalp and/or other parts of the –– Angiokeratoma: small red-violaceous papule body, which can be associated with various or plaque composed of dilated capillaries, skin diseases and either resolves or is permawhich can become crusty and bleed if accinent (scarring alopecia); alopecia areata is an dentally irritated; it can clinically mimic a autoimmune condition with increased amount melanoma. Angiokeratoma of Mibelli presof T cells in the hair matrix, presenting with ents with numerous reddish-blue warty papround bald patches containing broken exclaules on hands and feet, which can sporadically mation mark hairs (2–3 mm in length), which bleed. Angiokeratoma of Fordyce is most appear suddenly in hair-bearing areas, most commonly found on the scrotum, male and commonly on the scalp; androgenetic alopefemale genitalia, as small, symptomless red cia is a genetically predetermined disorder papules. Angiokeratoma circumscriptum due to excessive response to androgens, which refers to a vascular malformation presenting leads to progressive loss of terminal hair more as a cluster of reddish papules/plaques on the prominent in the vertex and frontotemporal leg or trunk. Angiokeratoma corporis difregions in the male and frontal hairline in fusum (Fabry disease) is a rare inherited disfemales; telogen effluvium refers to diffuse order caused by a deficiency of the

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alpha-galactosidase enzyme, leading to excessive deposits of glycosphingolipids in blood vessels and internal organs, where multiple angiokeratomas appear widespread at the lower trunk and groin area. Angiolymphoid hyperplasia with eosinophilia: nonmalignant lesion consisting of blood vessels surrounded by lymphocytes and eosinophils, presenting in younger subjects as clustered small, translucent nodules around the ear, hairline, or genital area; the lesions can be painful, itchy, or pulsatile and peripheral eosinophilia might coexist. Angioma: angiomas (cherry angioma) represent benign, vascular, mainly red-violaceous papules found on the trunk and occasionally extremities in middle-aged and elderly subjects; they are asymptomatic, can have various shapes (dome-like, polypoid papules), and usually increase in size and number with age. Angiosarcoma: rare, aggressive malignant tumor arising from the endothelium of blood or lymphatic vessels, mainly in the head-and-­ neck area of older people; it can also develop in patients with chronic lymphedema, radiotherapy patients, or patients exposed to environmental agents such as arsenic; cutaneous angiosarcoma usually presents with single or multiple, slow-spreading livid patches, which develop into nodules and plaques which easily bleed and ulcerate. Angular cheilitis: a common inflammatory condition of the oral commissures due to the presence of local irritants, xerosis, or infection (bacteria, yeast, virus). Antiphospholipid syndrome: acquired, systemic, autoimmune disorder which is characterized by the presence of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) leading to recurrent arterial or venous thrombosis, miscarriage, rashes, chronic headaches, and occasional seizures; can appear primarily or associated with other conditions such as lupus or drug intake; skin lesions include the presence of a livedo reticularis, leg ulcers, superficial thrombophlebitis,

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and vasculitis of small and medium-sized vessels [12]. Aphthous stomatitis: frequent recurrent oral, painful ulcers with fibrinous exudate surrounded by an erythematous halo, often idiopathic, induced by local trauma or associated with systemic disorders (Behcet’s disease, HIV), malnutrition (vitamin B12 deficiency, folate deficiency), infection, or drugs. Aplasia cutis congenital: rare physical finding that is present at birth showing as a solitary lesion on or near the vertex due to congenital absence of skin; the lesion usually consists of a circular or oval, well-demarcated defect or atrophic scar with alopecia, surrounded by a hair collar, and, in some cases, even ulcers reaching the bone. Apocrine hidrocystoma: rare, benign cystic tumor arising from apocrine sweat glands, presenting as a solitary, asymptomatic, dome-­ shaped, translucent papule or nodule most often in the head and neck area (especially the inner canthus of the eyelids). Ashy dermatosis: local dermal hypermelanotic disorder, characterized by well-­ circumscribed, oval, or irregular gray patches of the face, neck, or trunk in the absence of associated disease; the exact cause of the condition is unclear. Associations were described with contact allergies to cosmetics and hair dye, toxic effects of chemicals, viral infections, or adverse drug reactions. Atopic dermatitis: chronic, relapsing skin condition, usually arising in infancy, characterized by pruritic eczematous lesions, associated with a personal family history of allergic manifestations, arising due to a complex interaction of genetic and environmental factors; acute flares present with inflamed, erythematous, itchy, sometimes blistering or weeping patches and plaques; chronic atopic eczema presents with dry, thickened, lichenoid areas; recurrent staphylococcal infections may occur. Atrophia maculosa varioliformis cutis: rare idiopathic macular atrophy, often seen on the face of young subjects, which presents with

3  Dermatologic Concepts and Terminology

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the development of depressed lesions arranged presents in different types (solid, nodular, cysin a linear, reticulated or varioliform pattern tic, sclerosing, superficial, morpheaform, without previous trauma or inflammation. mixed) and is usually locally invasive into the –– Atrophoderma of Pasini and Pierini: rare surrounding tissue, metastasizing only in dermal atrophy condition presenting as exceptional cases (0.0028 and 0.55%) [13]; asymptomatic, well-defined, hyper- or usually, basal cell carcinomas appear as slow-­ hypopigmented depressed areas mainly growing, well-defined, translucent nodules or located on the trunk (most common site is papules with evident telangiectasia and facullumbosacral region); an association with mortative ulceration, as well as erythematous or phea and Lyme disease has been proposed; the sclerosing patches or plaques, depending on lesions present a sharp demarcation to the surthe histological subtype. rounding normal skin (“cliff-drop” borders). –– Basaloid follicular hamartoma: rare benign –– Atypical fibroxanthoma: dermal, fibrohistioneoplasm of the hair follicle, characterized by cytic tumor occurring mainly in sun-damaged the appearance of multiple brown papules of areas such as the head and neck in the elderly; the face, scalp, and trunk, which pose cosit usually presents as a solitary, red, dome-­ metic problems; the main differential diagnoshaped nodule with central bleeding, ulcersis is basal cell carcinoma. ation, or crusting, developing over months. –– Becker’s nevus: androgen-dependent lesion, often developing after puberty in male subjects, as a unilateral, asymptomatic, brown, B enlarging patch with hypertrichosis; it is more often localized on the chest, shoulders, or –– Bacillary angiomatosis: vasoproliferative, upper arms. reactive lesions of the skin, often seen in –– Behcet’s syndrome: chronic relapsing, autoimmunocompromised patients, usually caused immune vasculitis of unknown etiology, charby Bartonella henselae (responsible for cat acterized by recurrent painful oral and genital scratch disease), with possible systemic maniulcers, eye involvement (uveitis), and skin festations such as fever, malaise, and organ lesions (painful subcutaneous nodules); lesions (bones, soft tissue, liver, lymph nodes); patients can also present with sore throat, clinically, it presents with multiple, bright-red, muscular and joint pain, malaise, and weight disseminated papules and subcutaneous nodloss; the differential diagnoses to be ruled out ules and may be confused with Kaposi’s sarinvolve sexual infections and inflammatory coma or pyogenic granulomas. bowel diseases which can mimic Behcet’s –– Balanoposthitis: inflammation of the glans syndrome. penis and prepuce due to infectious (Candida, –– Benign lymphangioendothelioma: uncomStreptococcus, Staphylococcus aureus), irrimon, locally infiltrative lymphatic neoplasm, tant, or traumatic causes; can appear as erypresenting as a slow-growing reddish-­ thematous, sometimes erosive, patches; can be violaceous plaque or slowly enlarging associated with itching, burning sensations, as hematoma-­like patch mainly on legs and head well as a foul smell and unusual discharge. and neck area, which has to be differentiated –– Basal cell carcinoma: this is the most comfrom Kaposi sarcoma or angiosarcoma. mon nonmelanoma skin cancer and arises –– Black heel: asymptomatic condition of post-­ from the basal cell layer of the epidermis and traumatic intraepidermal hemorrhage, espeadnexal structures in patients with risk factors cially in young athletes, affecting the such as fair skin, chronic sun exposure, hisposterolateral aspect of the heel. tory of irradiation, history of skin cancer (per- –– Blastomycosis: a fungal infection caused by sonal or familiar), and use of tanning beds. It Blastomyces dermatitidis, transmitted by

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breathing in airborne spores from contaminated soil or wood, which can present with lung and skin involvement; patients usually present with respiratory, flu-like symptoms and local skin lesions consisting of papules, pustules, and subcutaneous nodules, which develop into ulcers over time, healing with scarring. Blue nevus: benign melanocytic lesion localized in the deep dermis, characterized by a dermal proliferation of melanocytes, appearing as a solitary, smooth-surfaced round or oval blue-black macule, papule, or plaque, usually benign; newly appearing blue nevi in older adults should be assessed and considered for excision to rule out melanoma. Blue rubber bleb nevus syndrome: a rare vascular disorder characterized by the presence of multiple cutaneous venous malformations, which appear as soft, compressible, red-violaceous-blue plaques and nodules, usually on the upper limbs, trunk, and soles; it can be associated with visceral lesions, most commonly involving the gastrointestinal tract, potentially leading to anemia due to bleeding or bowel obstruction [14]. Bowen’s disease: a type of in situ carcinoma, usually induced by UV light, human papillomavirus, or chemicals (arsenic), presenting as an asymptomatic, slowly enlarging, scaly, eczematous plaque commonly on the lower limbs or head and neck; it can also involve the glans penis, being known as erythroplasia of Queyrat; can potentially progress towards a squamous cell or Bowen carcinoma. Bowenoid papulosis: intraepithelial neoplasia, presenting as single or multiple, small, erythematous, brown or flesh-colored patches or papules in the genital area (penis shaft, labia) of sexually active adults; it can be associated with human papillomavirus; most cases of Bowenoid papulosis are benign, a small percentage can transform into squamous cell carcinoma of the penis/vulva. Branchial cleft cysts: abnormally formed congenital epithelial cyst of the lateral part of the neck, resulting from a failure of obliteration of the second branchial cleft in embryonic

development; clinically, they present as fistulae, cysts, sinus tracts, or cartilaginous areas often on the anterior neck or upper chest area. –– Bullous pemphigoid: common autoimmune, subepidermal blistering disorder, often seen in the elderly, as a result of autoantibodies developed against epidermal basement membrane structural proteins (hemidesmosomes); clinically, patients present with erythematous, urticarial, inflammatory plaques, followed by the development of round, tense bullae with serous or hemorrhagic fluid which develop into bleeding erosions once they rupture; it has a predilection for flexural areas and may occasionally involve mucous membranes; it can also be triggered by certain drugs, such as penicillamine, furosemide, and captopril. –– Burns: tissue damage resulting from heat, overexposure to the sun, radiation, and chemical or electrical contact; superficial, first-­ degree burns only affect the epidermis, causing local erythema, swelling, and pain, and usually heal without scarring; second-­ degree burns involve the epidermis and the superficial dermis, causing erythema, swelling, blistering, and severe pain and can heal with scarring; third-degree burns involve deeper structures of the skin, leading to local necrosis and numbness due to nerve destruction.

C –– Cafe-au-lait macule: well-circumscribed, asymptomatic, usually single, brownish patch that is most commonly seen on the trunk and extremities, caused by increased activation of melanocytes, usually present in early childhood; the presence of multiple macules (>6) is strongly suggestive of neurofibromatosis type I. –– Calcinosis cutis: deposition of calcium in the skin and subcutaneous tissue, presenting as firm, whitish papules, plaques, or nodules, singular or multiple; in time, lesions can become tender and ulcerate, discharging a chalklike material consisting of calcium phosphate; seen in various connective tissue dis-

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eases (dermatomyositis, systemic sclerosis, cutaneous lupus erythematosus), inherited diseases (Ehlers-Danlos syndrome, pseudoxanthoma elasticum), tumors (pilomatrixoma, cysts), or metastatic settings (paraneoplastic hypercalcemia, destructive bone disease). Candidiasis: cutaneous or mucous membrane infection caused by Candida albicans, appearing as an erythematous, diffuse eruption with peripheral satellite lesions mainly in moist skin folds (inner thighs, crural area, scrotum, underneath the breasts, diaper area in children); predisposing factors include an immunosuppressive state, diabetes, obesity, as well as use of systemic steroids or antibiotics. Cellulitis: infection of the dermis and subcutaneous tissue, mainly caused by group A streptococci appearing as erythematous, edematous, tender edema, mainly of the extremities. Chancroid: a sexually transmitted disease caused by the Gram-negative bacterium Haemophilus ducreyi, presenting with painful, necrotizing genital ulcers and inflammatory inguinal lymphadenopathy. Cheilitis granulomatosa (of Miescher): rare, idiopathic condition presenting with persistent, painless, nodular lip swelling due to local granulomatous inflammation in the absence of other causes such as inflammatory bowel disease, sarcoidosis, foreign-body reaction, or infection; cheilitis granulomatosa can also be a symptom which together with the presence of fissured tongue and recurrent partial or complete facial paralysis characterizes the Melkersson-Rosenthal syndrome. Chondrodermatitis nodularis helicis: a common, benign inflammatory condition of the skin and underlying cartilage of the helix or antihelix, associated with repeated pressure and compromised blood supply to the ear; it usually presents as a solitary, firm, oval-­ shaped, painful nodule with central crusting and surrounding erythema. Clavus: well-demarcated, hyperkeratotic painful area with a central core, often at foot level, appearing due to localized friction, pressure, or trauma.

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–– Clear-cell acanthoma: asymptomatic, benign epithelial skin tumor, mainly described on the lower legs, presenting as dome-shaped, erythematous, shiny plaque or nodule, with a discrete crusty scale in the periphery. –– Condyloma acuminatum: sexually transmitted disease of the genital area caused by human papillomavirus (HPV), presenting as singular or multiple filiform papules, large hyperkeratotic, verrucous plaques, or cauliflower-­like masses. –– Congenital melanocytic nevus: a benign proliferation of cutaneous melanocytes that are present at birth or develop shortly after; they usually present as round or oval patches or plaques with smooth, well-defined borders and different textures (papular, verrucous, cerebriform, rugose); small nevi are usually 5 mm, ill-defined borders, varying shades of color, etc.); while most melanocytic nevi are harmless, some might be removed to rule out melanoma. –– Melanoma: common skin cancer, with an increasing incidence worldwide, produced by the malignant transformation of melanocytes, occurring on the skin but also in other locations such as the gastrointestinal tract, brain, and eyes; risk factors include a positive family history of melanoma, personal characteristics (fair hair, blue eyes, Fitzpatrick skin types I– II), chronic sun exposure, presence of atypical mole syndrome, etc.; melanoma can present, depending on its growth pattern as superficial spreading melanoma, nodular melanoma, lentigo maligna melanoma, and acral lentiginous melanoma; usually, patients note a new or a change of color of a preexistent mole, an increase in diameter or height of the lesion, as well as an asymmetrical growth pattern; depending on the tumor depth, excision with safety margins and in some cases sentinel node biopsy are indicated to rule out lymphogenic spread. –– Melanonychia: melanin-derived, black or brownish pigmentation of the nail plate, which can be a normal finding in African people but can also appear as a consequence of local trauma, medications, nail infections, and local inflammation or due to the development of a nail matrix nevus; however, a newly developed melanonychia can also be a sign for the development of subungual melanoma, demanding a nail matrix biopsy to confirm the diagnosis; melanonychia usually appears as a diffuse or longitudinal nail band of different calibers.

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–– Melasma: localized, symmetrical, acquired hypermelanosis in sun-exposed areas, presenting with irregular brown, confluent, or disseminated macules, often in the facial area, associated with cosmetics, phototoxic drugs, hormonal status, etc. –– Melkersson-Rosenthal syndrome: rare condition of unknown etiology, presenting with the triad: recurrent orofacial swelling (cheilitis granulomatosa), relapsing facial paralysis, and lingua plicata (fissured tongue). –– Merkel cell carcinoma (MCC): rare, highly aggressive, neuroendocrine cutaneous malignancy associated with chronic UV exposure or the presence of polyomavirus, which usually arises in the elderly, in the head and neck area; clinically, MCC appears as rapidly growing, asymptomatic, firm, red-violaceous nodule in photoexposed skin. A sentinel node biopsy is indicated in patients with Merkel cell carcinoma because of the typical rapid progressive course, primarily metastasizing to the lymph nodes. –– Microcystic adnexal carcinoma: rare, malignant tumor developing from the sweat glands which commonly arises in the head and neck area, in sun-damaged skin, in areas of previous radiation, or immunocompromised patients; clinically, it presents as a slow-­ growing erythematous plaque or nodule which is often confused with a basal cell carcinoma. –– Milia: small cysts containing keratin, presenting as small white papules around the eyelids, cheeks, and forehead. –– Miliaria (rubra, cristallina): miliaria rubra appears as a consequence of ductal obstruction in the mid-epidermis, leading to the retention of sweat in the epidermis and dermis, which causes the appearance of irritated, erythematous pruritic papules; miliaria cristallina occurs in cases of ductal obstruction in the uppermost part of the epidermis, leading to sweat retention in the subcorneal layer, presenting with numerous clear, drop-like vesicles that rupture easily; miliaria typically occurs in the context of skin occlusion or hot environment.

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–– Molluscum contagiosum: viral infection caused by a poxvirus, characterized by centrally umbilicated papules on the face, extremities, trunk, or genital area, often in young children, sexually active adults, and HIV patients and with a self-limiting course. –– Mondor’s disease: a condition characterized by thrombophlebitis of the superficial veins of the anterior chest wall or other corporal regions presenting with the rapid development of a subcutaneous, tender, red, cord-like lesion that turns into a painless indurated fibrous band or cord with local skin retraction. –– Mongolian spot: characteristic benign, dermal melanocytic lesion of the sacral area, presenting as a uniform blue area of discoloration due to the arrested transdermal migration of melanocytes from the neural crest to the epidermis; lesions can also be located on the buttocks, flank, and shoulders. –– Morphea (localized scleroderma): connective tissue disorder of the skin and subcutaneous tissue, without organ involvement, presenting with localized smooth, shiny, sclerotic plaques with ill-defined borders and underlying induration; early lesions show a violaceous color and an active inflammatory border; according to the clinical presentation and depth of tissue involvement, morphea can be classified in various subtypes (plaque, linear, generalized, deep). –– Mycosis fungoides: indolent, cutaneous, CD4+ T-cell lymphoma of the skin, presenting with erythematous, scaly, pruritic, well-­ demarcated patches refractory to treatment; as the disease progresses, the patches develop into plaques and finally exophytic skin tumors, and subsequently systemic spread occurs, including lymphadenopathy and hepatosplenomegaly.

N –– Nail diseases (clubbing, striations, splinter hemorrhage, pitting, telangiectasia, etc.): encompass inflammatory, infectious, meta-

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bolic, malignant, or pigmentary disorders of the nail apparatus; some of the most common nail disorders are depicted in Table 3.6. Necrobiosis lipoidica: inflammatory, granulomatous disorder commonly seen in diabetic patients, appearing as scleroedematous, sharply demarcated, shiny, yellowish plaques in the pretibial area due to local collagen degeneration; central ulceration can appear in about 30% of patients. Neurofibroma: common nerve sheath tumor, presenting as skin-colored, soft papule or nodule, sometimes pedunculated, with a smooth surface; classically, neurofibromas present the “buttonhole invagination” sign: pressing on the lesion with the finger leads the tumor to fold in; plexiform neurofibromas generally appear on the trunk or extremities, are highly associated with neurofibromatosis type I, and are also prone to malignant transformation. Neurofibromatosis (NF): genetic disorder affecting the skin, nervous system, soft tissue, and bone, which can be classified as NF1 and NF2; NF1, also known as von Recklinghausen disease, is characterized by the presence of six or more cafe-au-lait macules, freckling of skin folds, Lisch nodules of the iris, and multiple neurofibromas of the skin; NF2 appears more rarely and is characterized by multiple tumors and lesions of the brain and spinal cord; the first symptom is usually hearing loss due to a tumoral growth compressing the auditory nerves. Nevi of Ota and Ito: congenital or acquired dermal melanocytic lesions of the face (nevus of Ota) and shoulders/neck (nevus of Ito), appearing as unilateral dark-bluish pigmentation of the cheek, eye (nevus of Ota), or chest region (nevus of Ito). Nevus flammeus (port-wine stain): pink-­ violaceous to red, sharply demarcated macules, sometimes with accompanying angiomatous nodules, most commonly seen in the head and neck area due to the presence of congenital capillary malformations at the cutaneous level; it can be associated with ocular abnormalities or Sturge-Weber syndrome

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Table 3.6  Common nail disorders in dermatological practice [1, 3, 24] Nail disorder Onychomadesis

Nail pitting

Trachyonychia True leukonychia Apparent leukonychia Koilonychia

Beau’s lines

Longitudinal melanonychia

Onychorrhexis Onychoschizia

Dorsal pterygium Nail-fold telangiectasias

Nail clubbing

Splinter hemorrhage Subungual hematoma Green nail Paronychia

Median nail deformity

Appearance Proximal nail plate detachment; when single nail involvement, there is often a traumatic cause; in the case of multiple nail involvement, a systemic cause may be present (chemotherapy, high fever, post-viral conditions) The presence of punctate depressions in the nail plate; this is seen in psoriasis together with oil-drop changes, onycholysis, subungual debris, or alopecia areata Thin, brittle, rough, sandpaper nails, associated with alopecia areata, lichen planus Whitish discoloration of the nail in a punctate or linear pattern (transverse or longitudinal) White appearance of the nail plate due to nail bed alterations, such as edema; secondary to drugs, systemic disorders, e.g., Terry’s nails Spooning of the fingernails, either idiopathic, familial, or associated with iron deficiency anemia; clinically, nails present elevated lateral distal edges and a depressed center The presence of transverse depressions of the nail plate, often secondary to trauma or systemic cause if more than one nail involvement (chemotherapy, fever, etc.) The presence of a longitudinal brown-black band due to several underlying causes: –  Racial predisposition (often present in darker skin types) –  Trauma (frictional, manicure, etc.) –  Drug intake (chemotherapeutics, psoralens) –  Post-inflammatory (lichen planus, onychomycosis, chronic radiodermatitis) –  Nevoid (nail matrix nevus) – Tumoral: nonmelanocytic (verrucae, Bowen’s disease, subungual keratosis), melanocytic (nail matrix melanoma) The presence of generalized nail thinning and ridging, present with normal aging or following a traumatic event The appearance of a lamellar splitting of the distal nail plate in several layers, which is common with aging or can appear as a consequence of chronic use of local irritants Development of a triangular extension of the proximal nail fold into the nail bed with consequent loss of local nail plate, often seen in lichen planus The presence of prominent capillaries and cuticular hemorrhage in patients with lupus erythematosus or Osler-Weber-Rendu syndrome; dilated capillary loops are also often seen in autoimmune disorders such as dermatomyositis or scleroderma The presence of watch-glass nails (>180-degree angle between the proximal nail fold and the nail plate) often seen in association with bronchopulmonary and cardiovascular disorders Thin longitudinal red-brown subungual lines secondary to trauma or in psoriasis The presence of a subungual red-brown-black patch, which moves distally as the nail grows and appears secondary to a local trauma Presence of blue-green coloration of the nail due to infection with Pseudomonas aeruginosa often associated with onycholysis Inflammation of the nail fold, with erythema, tenderness, and purulent drainage, secondary to bacterial (e.g., staphylococcal) or viral (herpetic whitlow) infections; chronic paronychia is characterized by chronic inflammation of the proximal nail fold with cuticle loss The presence of multiple transverse grooves of the nail plate with a central longitudinal depression due to mechanical manipulation of the area above the matrix (continued)

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Appearance Hypoplasia of the fingernails and triangular lunulae Tender inflammation of the lateral nail fold with overgrowth of granulation tissue, secondary to malalignment or drugs Extreme curling and thickening of the nail plate leading to a hornlike appearance, often in the elderly, at the level of the first toe Yellow coloration of multiple fingers and/or toenails associated with chronic lymphedema and bronchopulmonary disorders Benign Malignant Keratoacanthoma Pyogenic granuloma Bowen’s disease Periungual fibroma Melanoma in situ Myxoid cyst Melanoma of the nail matrix Warts Squamous cell carcinoma Melanocytic nevi Verrucous carcinoma Subungual exostosis Glomus tumor Onychopapilloma Onychomatricoma

when localized on the forehead, eyelids, and size of the nose. –– Nevus sebaceous: congenital hamartoma of the pilosebaceous follicular unit, mostly occurring on the scalp, forehead, face, or neck, presenting as a solitary, smooth, yellow-­ orange hairless patch often oval or linear in shape, which in time can become verrucous; sometimes other tumors, such as trichoblastomas, eccrine poroma, adnexal carcinoma, or basal/squamous cell carcinoma, may develop within the nevus. –– Nocardiosis: infectious, suppurative, or granulomatous disease caused by aerobic soil saprophytes, presenting with cutaneous, pulmonary, and disseminated forms; cutaneous nocardiosis is characterized by the presence of skin or subcutaneous abscesses; lymphocutaneous syndrome presents with a primary pyoderma lesion and multiple lymphatic nodules (sporotrichoid nocardiosis); in some cases, an actinomycetoma can develop, presenting as a nodule that suppurates, spreads along the fascia, and develops multiple draining fistulae. –– Nummular eczema: scattered, well-defined, nummular erythematosquamous plaques associated with staphylococcal infections, local injuries, xerosis cutis, varicose veins, contact dermatitis, etc.

O –– Onycholysis: common nail disorder involving the separation of the nail plate from the nail bed, typically presenting as a well-defined area of whitish-opaque nail; can be either idiopathic or secondary to local trauma, inflammatory skin disorders (psoriasis), nail infections, etc. –– Onychomatricoma: rare, benign, fibroepithelial tumor of the nail matrix, presenting as a slow-growing, painless, thickened nail plate with proximal splinter hemorrhage, overcurvature of the nail plate, and fingerlike projections penetrating the nail plate, leading to the appearance of “woodworm“ cavities under the nail plate’s free margin. –– Onychomycosis: common fungal infection of the nail, causing discoloration, thickening, and onycholysis, mainly caused by T. rubrum and T. interdigitale; it can present with subungual hyperkeratosis, distal onycholysis, superficial white patches on the nail plate, or complete destruction of the nail apparatus and may affect one or more toe or fingernails. –– Onychopapilloma: benign tumor of the nail matrix, presenting as a longitudinal streak (red-erythronychia, brown-melanonychia, white-leukonychia) from the lunula to the nail tip; the distal nail may present with onycholy-

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sis, and occasionally subungual keratosis or splinter hemorrhage can appear. –– Osler-Rendu-Weber syndrome: autosomal-­ dominant condition characterized by multiple mucocutaneous telangiectasias, which can lead to the nose, gastrointestinal bleedings, and iron deficiency anemia; since these arteriovenous malformations can develop in numerous organs, patients can suffer from life-threatening complications. ––

P –– Paget’s disease (of the breast, extra-­ mammary): Paget’s disease of the breast is a rare disease more prevalent in postmenopausal women, presenting as an eczematoid, erythematous, crusted lesion with or without infiltration and inversion of the nipple, often –– associated with intraductal, in situ, or invasive neoplasms; extra-mammary Paget’s disease is a rare adenocarcinoma originating from the skin or appendages in areas with apocrine –– glands, primarily presenting at vulvar level, –– perianal region, scrotum, penis, and axilla; it is usually an intraepithelial lesion, not associated with distant cancer, and presents clinically as erythematous plaques, with indolent growth, well-defined borders, and superficial fine scales. –– –– Paronychia: soft-tissue infection around the nail, presenting as painful, purulent erythematous swelling of the surrounding tissue and nail fold, most commonly caused by staphylococcal infections; recurrent episodes can be –– caused by herpes simplex infections (herpetic whitlow). –– Pediculosis: parasitic skin disease caused by Pediculus humanus capitis, humanis, or pubis, which can be found on the occiput or behind the ears or genital area; other local signs include tiny hemorrhagic papules (bites), crusted areas, and secondary –– impetiginization. –– –– Pemphigus vulgaris: rare, chronic, blistering autoimmune disease of the skin and mucous membranes divided into various subtypes

(pemphigus vulgaris, vegetans, foliaceus, paraneoplastic); pemphigus vulgaris is caused by the development of autoantibodies binding to desmoglein 1 and 3 within the epithelial skin layer leading to intraepidermal blistering; clinically, it presents with multiple oropharyngeal mucosal lesions preceding a generalized bullous eruption; the blisters are fragile and rupture easily, leaving painful, denuded, crusted erosions. Peutz-Jeghers syndrome: autosomal dominant condition presenting with gastrointestinal polyposis (small intestine), mucocutaneous hyperpigmentation (hyperpigmented macules around the mouth, lips, eyes, buccal mucosa), and cancer predisposition (colorectal, gastric, ovarian, breast cancer); the presence of polyps can result in chronic bleeding, anemia, and also bowel obstruction. Photodermatosis: disorders caused by an abnormal reaction to UV rays, which can be phototoxic, photoallergic to known photosensitizers, or idiopathic. Pilar cyst: see trichilemmal cyst. Pilomatricoma: benign hair follicle tumor of young children and adults, presenting as single skin-colored or purplish papule or nodules, usually of the head and neck area; can have a lobular appearance and be firm upon palpation when there is calcification within. Pityriasis alba: common skin disorder in children, often associated with eczema, presenting with ill-defined, scaly, hypopigmented patches of the skin, trunk, or upper extremities. Pityriasis rubra pilaris: rare, chronic inflammatory skin disorder characterized by the presence of hyperkeratotic follicular papules and/or erythematosquamous plaques, which can lead to erythroderma, as well as palmoplantar hyperkeratosis; between extensive erythematous areas, small islands of non-affected skin, “nappes-claires,” are characteristic. Pityriasis versicolor: see tinea versicolor. Pityriasis rosea: common, self-limiting, symmetrical skin eruption, consisting of multiple, oval-shaped, salmon-colored scaling patches (collarette scaling) distributed along skin lines

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in a dropping pine-tree fashion (trunk); an initial lesion (herald patch) precedes the eruption by 1–2 weeks. Polyarteritis nodosa: vasculitis of medium and small arteries, presenting with livedo reticularis and ulcerations of digits and shins, an asymmetric polyarthritis, and sometimes systemic inflammation signs (myalgia, fever). Polymorphous light eruption: symmetrical, pruritic erythematous papules, plaques, or vesicles, mainly on the face, upper back, chest, and extremities, following exposure to UV light. Pompholyx: type of eczema of the hands and feet presenting with multiple, itchy blisters or bullae mainly on the side of the fingers, palms of the hands, and soles, often associated with hyperhidrosis and atopic eczema. Poroma: benign adnexal tumor originating in the terminal sweat gland duct, presenting as erythematous or flesh-colored nodules of the palms and soles. Porphyria cutanea tarda: the most common form of cutaneous porphyria, appearing due to a reduced hepatic uroporphyrinogen decarboxylase enzyme activity, resulting in excess porphyrins and cutaneous photosensitivity, leading to the appearance of blistering, milia, scarring, cutaneous fragility, hypertrichosis, and premature aging in sun-exposed areas; the condition may be precipitated by exogenous factors, such as alcohol, iron overload, and sun exposure, and a co-association with hepatitis C and HIV is also described. Progeria (Hutchinson-Gilford progeria syndrome): genetic condition characterized by the onset of accelerated aging already beginning in childhood; the affected children present with a characteristic facial appearance (prominent eyes, beaked tip nose, protruding ears) as well as hair loss, loss of the subcutaneous fat, prematurely aged skin, and small stature; while it does not affect the intellectual development of the patients, patients experience severe atherosclerosis, posing them at risk for stroke or cardiovascular incidents at a young age [25].

–– Prurigo: chronic disorder characterized by the presence of multiple itchy papules and nodules, which can appear primarily or secondarily after scratching; prurigo simplex is characterized by the appearance of symmetrically distributed erythematous itchy papules and occasionally blisters; nodular prurigo presents with numerous firm, warty nodules with central ulceration or crusts; it can be associated with conditions such as atopic eczema, bullous pemphigoid, chronic renal failure, Hodgkin lymphoma, and diabetes. –– Pseudocyst of the auricle: benign condition characterized by an asymptomatic cystic lesion of the lateral or anterior side of the auricle (scaphoid or triangular fossa), occasionally associated with traumatic events. –– Pseudofolliculitis barbae: chronic inflammatory disorder of hair-bearing areas in patients shaving regularly presenting as painful perifollicular papules and pustules, sometimes complicated by keloid and abscess formation. This entity has also been linked to hidradenitis suppurativa. –– Pseudoxanthoma elasticum: hereditary connective tissue disorder leading to swelling and fragmentation of the elastic fibers of the mid-­ dermis, presenting as thickened, yellowish plaques and sagging of the skin on the neck, axillae, and flexural areas, resembling “plucked chicken” skin; can affect not only the skin but also blood vessels and eyes, patients being at risk of hypertension, ischemic heart disease, as well as gastrointestinal, cerebral, and retinal hemorrhage. –– Psoriasis: chronic skin disorder characterized by excessive proliferation of keratinocytes, leading to the appearance of sharply demarcated, thickened, erythematous plaques with silvery scales adherent to the psoriatic lesions, which bleed if removed (Auspitz sign); it primarily affects the elbow and knee region, the intergluteal fold, and the scalp; psoriasis presents in many forms: chronic stationary, guttate, pustular, and erythrodermic and may involve nails and joints; psoriatic lesions can also develop at the site of local trauma (Köbner phenomenon).

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–– Purpura: coloration of the skin and mucous bite, presenting with fever, headache, as well membranes due to hemorrhage from small as a macular rash, which progresses to papblood vessels due to different causes (platelet, ules, petechiae, and ecchymosis. The rash coagulation, or vascular disorders); the extravusually begins on the ankles and wrists and asated blood usually changes color over a few then spreads to the palms and soles, limbs, and weeks from purple, brown, and yellow to trunk. green and blue; palpable purpura is usually –– Rosacea: chronic skin condition, characterseen in vasculitis, due to the local inflammaized by facial flushing, erythema, papules, and tion of small blood vessels. pustules of the face, knowingly flaring up after –– Pyoderma gangrenosum: uncommon, ulcerhot drinks, alcohol, stress, sun exposure, cerative cutaneous condition of uncertain etioltain medications (simvastatin, ACE inhibiogy, initially presenting as a small pustule that tors), etc.; typical stages include initial quickly develops towards a painful ulcer with centro-facial erythema, telangiectasia, and dark, irregular margins; it is often associated flushing; then the appearance of a papulopuswith other conditions such as inflammatory tular eruption; and finally the phymatous bowel disease, arthritis, and autoinflammatory phase, presenting with thickened skin with syndromes. prominent pores affecting the nose (rhino–– Pyogenic granuloma: benign vascular lesion phyma), but also chin, forehead, and eyelids; of the skin and mucous membranes, due to an ocular involvement, consisting of conjunclocal capillary proliferation secondary to tival injection, blepharitis can appear in about trauma, presenting as usually small, dome-­ 40% of cases [26]. shaped, sometimes ulcerative, friable papules/ –– Rubella: viral illness caused by the rubella nodules. virus, presenting with maculopapular erythema on the face and spreading to the trunk, with cervical and occipital lymphadenopathy; R during pregnancy, especially in the first trimester of gestation, it can lead to congenital –– Raynaud’s phenomenon: vasospastic disoranomalies of the fetus. der, producing an exaggerated response to –– Rubeola (measles): childhood exanthema, cold temperatures or stress, resulting in trancaused by the Morbillivirus, transmitted by sient digital ischemia; patients experience a airborne droplets, presenting as an erythematriphasic discoloration of fingers and toes (initous maculopapular eruption beginning behind tially intense blanching of the fingers due to the ears, progressing to the forehead and neck, vasoconstriction, then bluish coloration due to and then spreading to the face, trunk, and cyanosis, and finally red due to hyperemia and extremities. A few days later, the rash fades local reperfusion). with local desquamation; enanthema (Köplik –– Reiter syndrome: asymmetrical seronegative spots) usually appears on the buccal mucosa spondyloarthropathy mainly affecting the before the cutaneous rash. lower extremities and associated with uveitis, cervicitis, inflammatory eye disease, and mucocutaneous lesions; the physical examina- S tion presents with hyperkeratotic lesions on soles, toes, and hands (keratoderma blenoragi- –– Sarcoidosis: multisystem granulomatous discum), resembling psoriasis, as well as localorder usually involving the lungs and the ized erythema and scaling of the penis lymph nodes, but also skin in 25% of cases; (circinate balanitis). cutaneous sarcoidosis presents with erythema –– Rocky Mountain spotted fever: bacterial nodosum (tender subcutaneous nodules on infection (Rickettsia rickettsii) spread by tick the shins), sometimes accompanied by arthri-

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tis and hilar lymphadenopathy (Löfgren syndrome), as well as disseminated brownish papules and nodules; it can also infiltrate old scars (scar sarcoidosis) or present as lupus pernio, infiltrative plaque-like lesions of the nose, cheeks, or ears. Scabies: contagious disease caused by a mite (Sarcoptes scabiei), generally associated with poor living conditions, presenting with scattered, inflamed excoriated papules and vesicles on the hands, wrists, buttocks, scrotum, penis, breast, axillae, and knees, accompanied by intense nocturnal pruritus. Scarlet fever: bacterial infection of the childhood, presenting with sudden fever associated with a sore throat, lymphadenopathy, red strawberry tongue, and a characteristic rash developing 12–48  h after the fever episode; clinically, the rash begins in the infra-­auricular area, spreading to the neck, chest, flexures, and the rest of the integument; the skin has a rough sandpaper-like appearance. Schwannoma: benign tumor which develops from the Schwann cells in the peripheral nervous system or cranial nerves; clinically, schwannomas present as 1–2  cm large, firm nodules in the head and neck area; multiple schwannomas are usually associated with neurofibromatosis. Sebaceous carcinoma: rare, aggressive form of skin cancer arising from the meibomian glands located at eyelid level, presenting as small, erythematous, firm, slowly growing nodules of the lower or upper lid, which can spread onto the conjunctiva, and in case of late diagnosis also to the regional lymph nodes and parotid gland; extraocular lesions can present as firm nodules of different sizes. Seborrheic dermatitis: relapsing inflammatory disorder with a predilection for areas rich in sebaceous glands (scalp, forehead, perinasal area, eyebrows, presternal, interscapular area), presenting with localized erythematous patches with yellowish, greasy scales. Seborrheic keratosis: common benign skin lesions, developing on normal skin as one or more, sharply defined, verrucous, “stuck-on,” lightly pigmented plaques in middle-aged or elderly people.

–– Shingles: see herpes zoster. –– Sjögren’s syndrome: autoimmune disorder of the tear and saliva glands, presenting with dry mouth, dryness of nasal passages, painful burning sensation around the eyes, as well as swelling of the involved glands; primary Sjögren’s syndrome usually occurs in the absence of other diseases, while secondary Sjögren disease can be associated to other rheumatologic conditions, most often lupus erythematosus or rheumatoid arthritis. –– Spider angioma: solitary skin lesion consisting of a dilated dermal arteriole that communicates with a network of superficial capillaries, clinically resembling the spindly legs of a spider emerging from a central-­ feeding arteriole; the angioma compresses easily upon direct pressure and refills following pressure release. –– Sporotrichosis: cutaneous fungal infection caused by Sporothrix schenckii, usually found on soil and vegetation; the cutaneous lesions (erythematous, painless nodules which ulcerate) develop in a sporotrichoid spread from the site of scratch along the lymphatic channels; patients can also present with inflammatory arthritis of one or more joints, as well as organ involvement (eye, larynx, prostate, brain) in case of disseminated disease. –– Squamous cell carcinoma: common nonmelanoma skin cancer arising from epidermal keratinocytes, mainly in chronically sun-­ damaged skin of older subjects, but also in genodermatoses (xeroderma pigmentosum, albinism), following exposure to arsenic, immunosuppressive state, chronic wounds, or organ-transplant recipients; clinically, they present as scaly, hyperkeratotic, ulcerated plaques or nodules especially on the face, lips, ears, hands, forearms, and lower legs. –– Staphylococcal scalded skin syndrome (SSSS): blistering skin condition usually occurring in association with a staphylococcal infection due to the release of exfoliative toxins, presenting as a macular scarlatiniform eruption of the face and flexures (axilla, groins) extending to the remaining integument, with accompanying conjunctivitis; traction pressure on intact skin causes bulla formation (Nikolsky

3  Dermatologic Concepts and Terminology

––

––

––

––

––

––

––

sign), and the epidermis can be shred off in thin sheets, leaving large erosions. Stasis dermatitis: inflammatory skin condition of the lower extremities, commonly associated with chronic venous insufficiency, presenting with chronic edema, erythematous-­ scaly patches (eczematoid dermatitis), brown hyperpigmentation due to hemosiderin deposits, as well as multiple varicose veins. Stevens-Johnson syndrome (SJS): rare, immune-mediated skin disorder resulting in skin blistering and extensive epidermal detachment, usually triggered by medications (antibiotics, antiepileptics, etc.); patients present with flu-like symptoms as well as painful vesiculobullous lesions, which rapidly denude to form skin erosions, similar to burns; mucous membrane involvement is also present, and patients present with oral and genital ulcers and/or severe conjunctivitis; when more than 30% of the body surface is involved, the condition is referred to as toxic epidermal necrolysis (TEN) [27]. Striae: streak-like skin lesions mostly found on the abdomen, breasts, and thighs in pregnant patients, during long-term steroid therapy, Cushing’s syndrome, after rapid weight gain, etc. Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE): symmetrical maculopapular erythema or plaques of the gluteal and intertriginous areas following exposure to specific drugs; mucosal lesions are not present. Syphilis: sexually transmitted treponemal disease, caused by Treponema pallidum, characterized by a primary skin eruption (painless chancre on genitalia, mouth, anus), secondary eruption involving skin and mucous membranes (maculopapular lesions of palms and soles, mucous patches, generalized lymphadenopathy), and late lesions of the skin, organs, bone, and central nervous and cardiovascular system. Syringoma: benign adnexal tumor arising from ductal elements, presenting as multiple, skin-colored, small dermal papules often seen at lower eyelid and cheek level. Systemic sclerosis: an autoimmune inflammatory condition resulting in extensive fibrosis

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and vascular abnormalities, affecting the skin, lungs, gastrointestinal tract, heart, and kidneys; clinically, it presents with progressive thickened, hard skin of the fingers and toes (sclerodactyly) but also of the rest of the integument, nail-fold abnormalities, as well as fibrosis of internal organs and vascular damage.

T –– Telogen effluvium: see alopecia. –– Terry’s nails: apparent leukonychia, presenting with a whitish opacification of the entire nail plate, with a narrow band of normal, pink nail bed at the distal nail border and obliteration of the lunula; present in systemic diseases, such as chronic congestive heart failure, liver disease, renal failure, and metastatic cancer. –– Thrombophlebitis: inflammatory thrombosis of subcutaneous veins, presenting with erythema, tenderness, and a palpable cord after local trauma to preexisting varices, intravenous cannulation, infection, and deep vein thrombosis of hypercoagulable state. –– Thyroglossal duct cysts: most common anomaly of the neck, presenting as skin-­ colored, soft, cystic nodules in or lateral to the midline in the area from the hyoid bone to the suprasternal notch; when attached to the hyoid bone, the cyst tends to move upward when swallowing. –– Tinea (barbae, facie, capitis, corporis, cruris, pedis): superficial dermatophyte infection of various skin areas, presenting as annular, erythematous patches with active, scaly borders with some crusting and subsequent development of satellite lesions. –– Tinea versicolor: fungal skin infection caused by the lipophilic yeast (Malassezia furfur), presenting as numerous circular, scaly macules of different colors mainly on the trunk (hyperpigmented, hypopigmented). –– Toxic epidermal necrolysis (TEN): acute, potentially fatal skin reaction, mostly following medication use (sulfonamides, penicillin, NSARs, anticonvulsants) or infection (mycoplasma, CMV); symptoms include fever, flu-­

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––

––

––

––

––

––

like symptoms, as well as a tender erythematous skin rash starting on the trunk and extending rapidly over few hours/days onto the face and extremities; lesions can be macular (diffuse confluent maculae), purpuric, targetoid (as in erythema multiforme), or blisters, which merge and eventually lead to a superficial skin detachment, exposing the underlying dermis; TEN patients have a positive Nikolsky sign; mucosal involvement is usually prominent, involving eyes (conjunctivitis, uveitis), lips (cheilitis, stomatitis), oral mucosa (mouth ulcers), pharynx, genital area, and gastrointestinal tract [27]. Trichilemmal cyst: keratin-filled scalp cyst originating from the outer hair root sheath presenting as singular or multiple nodules (inherited as an autosomal dominant trait) of the scalp without a central pore, unlike the epidermoid cysts; may become tender when inflamed. Trichoblastoma: small, benign hair follicle tumor, presenting as skin-colored or brown papules or nodules on the face and scalp of middle-aged people; may rarely transform into malignant trichoblastic carcinoma, which can be locally aggressive but seldom metastasize. Trichoepithelioma: benign skin lesions originating from hair follicles, mostly seen in the scalp, nose, forehead, and upper lip area; can appear as part of Brooke-Spiegler syndrome; clinically, they present as tiny, firm, round papules/nodules resembling syringomas or milia. Trichofolliculoma: benign, small solitary, flesh-colored nodule of the face or scalp in adults, developing from the hair follicle. Trichotillomania: traumatic alopecia due to traction or pulling of the hair, mainly involving the frontoparietal area. Tuberous xanthomas: yellow-orange, sharply defined nodules usually seen over the knees and elbows in patients with hypercholesterinemia or other lipid disorders.

U –– Ulerythema ophryogenes: form of keratosis pilaris with scar-like follicular depressions and loss of hair at eyebrow level; it

usually presents with keratotic follicular papules of the eyebrows, forehead, and cheeks, leading in time to local atrophy and permanent hair loss. –– Urticaria pigmentosa: most common type of cutaneous mastocytosis, characterized by the development of brown patches in early infancy, which gradually progress in number over time and involve any part of the body; rubbing the lesions leads to the development of blisters (positive Darier sign); in time, the lesions become less itchy and tend to disappear; when developing in adults, urticaria pigmentosa tends to persist for a longer time and can be associated with systemic symptoms (flush, anaphylactic shock, diarrhea, and gastrointestinal bleeding), suggesting the presence of a systemic mastocytosis. –– Urticaria: pruritic rash manifesting with the appearance of short-lived, well-circumscribed, pink wheals changing in shape and size following exposure to a triggering agent (foods, drugs, infections, contact, etc.).

V –– Varicella: common viral illness caused by the varicella-zoster virus, presenting with an acute onset of a generalized vesicular rash (disseminated papules and vesicles on erythematous base—“dew drops on a rose petal appearance”) with centripetal distribution and fever. –– Varicose veins: dilated networks of subcutaneous veins resulting from valvular incompetence, presenting as marked, dilated, tortuous linear strings, often accompanied by stasis dermatitis and hyperpigmentation due to chronic venous insufficiency. –– Vasculitis: inflammation of cutaneous blood vessels (capillaries, venules, arterioles, lymphatics) due to different insults (bacterial or viral infections, activation of antibodies or complement, medication), which can sometimes be associated with systemic symptoms; the clinical presentation depends on the size of inflamed blood vessels: small-vessel vasculitis (leukocytoclastic vasculitis) presents with palpable pur-

3  Dermatologic Concepts and Terminology

pura, medium-vessel vasculitis with nodules and livedo reticularis, while large-­vessel vasculitis mainly consists of systemic symptoms. –– Venous lake: localized vein dilation of an endothelium-lined blood vessel, often seen on the ventral surface of the tongue, lower lip, and buccal mucosa, presenting as bluish papules/plaques which blanch under pressure and can become firm if thrombosed. –– Venous leg ulcers: full-thickness ulcers with irregular borders and areas of granulation, often occurring on the lower extremities above or over the malleoli, occurring due to venous hypertension caused by incompetent valves or obstructed veins; patients with venous ulcers also present with edema, skin color changes, scaly eczematous changes (stasis dermatitis), plaques of atrophic sclerosis (atrophie blanche), as well as worsening pain after prolonged standing. –– Vitiligo: autoimmune, acquired loss of epidermal pigmentation due to the destruction of epidermal melanocytes, presenting with gradually progressive, hypopigmented and depigmented, sharply demarcated patches often in sun-exposed areas, genitalia, and intertriginous areas.

W –– Warts: benign, epidermal neoplasms caused by human papillomavirus, presenting as solitary or multiple, keratotic plaques with a rough surface and black dots (thrombosed capillaries).

X –– Xanthelasma: solitary or multiple, soft yellow-­orange papules or plaques often seen on the upper medial eyelids, often in hyperlipidemic patients. –– Xanthoma: accumulation of lipids in the skin and subcutaneous tissue in patients with hyperlipidemia, endocrine or metabolic disorders, presenting as yellow-red dome-shaped papules.

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–– Xerosis: dry, rough-appearing scaly skin with associated pruritus, as a sign of aging, systemic illness (renal failure, malnutrition, etc.), keratinization disorders, atopic dermatitis, and failure of skin hydration.

Common Dermatologic Signs In the dermatological field, many clinical signs which are highly pathognomonic for certain disorders have been observed and documented throughout time; these signs either appear de novo or can be elicited by the physician. The following paragraphs illustrate some of the most commonly used signs for diagnosing various skin conditions or narrowing down the list of differential diagnoses [28, 29]. –– Auspitz sign: the appearance of superficial punctate bleeding after removal of the psoriasis scale by scraping or scratching. –– Butterfly rash: erythema of the malar eminence and nasal bridge, classically present in lupus erythematosus patients. –– Butterfly sign: present in patients with prurigo nodularis, referring to the sparing of the mid-scapular region from scratching, as they are not able to reach the area. –– Buttonhole sign: invagination of neurofibromas into the underlying subcutis using digital pressure and reappearance after pressure release in type I neurofibromatosis. –– String of pearls sign: typical in linear IgA dermatosis in childhood, with peripherally/ annularly situated vesicles resembling a string of pearls. –– Crowe’s sign: axillary freckling in patients with type I neurofibromatosis. –– Darier sign: whealing, erythema, and localized pruritus in patients with mastocytosis and urticaria pigmentosa, following rubbing of the lesions. –– Dimple sign: used for the diagnosis of dermatofibromas; the lateral compression of the lesion with the thumb and index finger leads to a depression of the lesion due to its attachment to the subcutaneous fat.

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–– Dirty neck sign: the presence of reticulate pigmentation of the neck in patients with chronic atopic dermatitis, secondary to melanin incontinence. –– Drip sign: in patients with dermatitis artefacta produced by corrosive liquids; the burn lesions correspond to the areas of the dripping of the substance. –– Exclamation mark hair: the proximal tapering of hair seen in alopecia areata. –– Gottron’s sign: the presence of symmetrical, violaceous erythema over the knuckles, hips, knees, and ankles in patients with dermatomyositis. –– Heliotrope sign: violaceous erythema of the periorbital area in patients with dermatomyositis. –– Hertoghe’s sign: the lack of the outer third of the eyebrows in patients with atopic dermatitis (minor diagnosis criteria) , alopecia areata, hypothyroidism, trichotillomania, etc. –– Hutchinson’s sign: the periungual extension of pigmentation onto the proximal or lateral nail fold, often seen in acral malignant melanoma. –– Hutchinson’s nose: the presence of vesicles on the tip of the nose in patients with herpes zoster, suggesting the affection of the nasociliary branch and potential ocular involvement. –– Leser-Trelat sign: sudden eruption of multiple seborrheic keratoses on the body, often secondary to an internal malignancy (adenocarcinoma, lung cancer, melanoma, etc.). –– Nikolsky sign I: the ability to split the epidermis by a shearing force (lateral pressure with the finger) in areas distant from the lesion, indicating a plane of cleavage in the skin at the dermal-epidermal junction; seen in Stevens-­ Johnson syndrome, toxic epidermal necrolysis, pemphigus vulgaris, and pemphigus foliaceus. –– Nikolsky sign II: lateral pressure on the edge of the blister may spread the blister into clinically unaffected skin; often seen in bullous pemphigoid. –– Oil-drop sign: yellow-red discoloration and areas of onycholysis in the nail bed, often seen in psoriasis with nail involvement.

D. Crisan and M. Crisan

–– Pathergy sign: the creation of new lesions or worsening of existing ones by superficial trauma such as skin testing and injections, often seen in patients with pyoderma gangrenosum and Behcet’s disease. –– Shawl sign: the presence of a symmetrical, confluent, violaceous erythema on the posterior shoulders and neck in patients with dermatomyositis. –– Slapped-cheek sign: seen in children with the fifth disease as erythematous, confluent plaques of the cheeks. –– Tent sign: often seen in patients with pilomatrixoma: upon stretching the overlying skin, the lesion appears as angulated, conferring a tentlike appearance (due to the presence of calcification areas within the lesion). –– Ugly duckling sign: refers to the observation that a nevus that does not resemble the other nevi of a patient should be suspicious of melanoma. –– V sign: erythema induced by photosensitivity in the V-area of the upper chest in patients with dermatomyositis.

 ifferential Diagnosis of Skin D Disorders According to Lesion Morphology and Affected Region The integumentary system represents the largest organ of our body, covering the entire external body surface and being completely exposed to external environmental factors; therefore, unlike many other specialties which deal with more or less than 50–60 commonly seen conditions, the dermatological field encompasses a very vast range of disease patterns affecting the skin and its appendages (ca. 1000–2000 conditions) [30]. Since most of the dermatological conditions are fully disposed at the body surface and can be characterized by well-described primary or secondary skin lesions (see “Initial Approach to Skin Lesions”), many skin disorders can be diagnosed, and the differential list is narrowed down following an adequate description of the skin changes in our patients; this can sometimes, how-

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3  Dermatologic Concepts and Terminology

ever, be overwhelming for colleagues of other disciplines. For this purpose, this chapter intends to provide an overview of the most common skin disorders according to their morphology and topographic distribution, which can help us ori-

ent the diagnosis in our patients (Table  3.7, Fig.  3.4). Nevertheless, it should be noted that many dermatologic lesions evolve during the course of the disease; hence, a particular condition might present different morphological aspects in time, which is why several conditions

Table 3.7  Distribution of common skin conditions according to lesion morphology [1, 9] Macules

Hypopigmented –  Halo nevus –  Idiopathic guttate hypomelanosis – Leprosy –  Nevus achromicus –  Post-inflammatory macules – Sarcoidosis –  Tinea versicolor –  Tuberous sclerosis – Vitiligo

Papules

Isolated papules –  Accessory nipple/accessory tragus –  Acrochordon (skin tag) –  Actinic keratosis –  Adenoma sebaceum – Angiofibroma/angiokeratoma –  Arthropod bite –  Basal cell carcinoma –  Blue nevus –  Cherry angioma –  Chondrodermatitis nodularis helices – Dermatofibroma –  Eccrine poroma – Hemangioma – Keratoacanthoma – Melanoma – Milia –  Molluscum contagiosum – Neurofibroma –  Nevi (dermal) –  Pearly penile papule –  Pseudoxanthoma elasticum –  Pyogenic granuloma –  Sebaceous hyperplasia –  Seborrheic keratosis –  Squamous cell carcinoma –  Syringocystadenoma papilliferum – Syringoma – Trichoepithelioma/trichofolliculoma –  Venous lake – Warts

Hyperpigmented Erythematous –  Becker’s nevus –  Drug eruptions –  Cafe-au-lait spots – Juvenile rheumatoid – Ephelides arthritis – Erythrasma –  Rheumatic fever –  Junction nevus –  Secondary syphilis –  Lentigo (maligna) –  Viral exanthema – Melasma –  Mongolian spot – Post-inflammatory macules – Purpura –  Stasis dermatitis –  Tinea nigra Papular eruptions –  Acne vulgaris –  Acneiform rosacea –  Bacillary angiomatosis –  Cholinergic urticaria –  Condyloma acuminatum –  Darier disease – Dermatomyositis –  Dermatosis papulosa nigra –  Drug eruption – Eczema –  Flat warts – Folliculitis –  Granuloma annulare –  Kaposi sarcoma –  Keratosis pilaris –  Lichen (planus, nitidus) –  Lupus erythematosus – Lymphoma – Miliaria –  Molluscum contagiosum – Neurofibromatosis – Pediculosis –  Perioral dermatitis –  Polymorphous light eruption –  Pruritic urticarial papules, plaques of pregnancy – Sarcoidosis – Scabies –  Secondary syphilis –  Urticaria pigmentosa – Vasculitis –  Viral exanthema –  Xanthoma (isolated, eruptive) (continued)

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62 Table 3.7 (continued) Pustules

Plaques

–  Acne vulgaris – Candidiasis –  Dermatophyte infections –  Drug eruptions (e.g., acute generalized exanthematous pustulosis) – Dyshidrosis – Eczema –  Eosinophilic folliculitis –  Erythema toxicum neonatorum – Folliculitis – Furunculosis –  Herpes simplex, herpes zoster –  Hidradenitis suppurativa – Impetigo –  Langerhans cell histiocytosis –  Neonatal pustulosis –  Pseudofolliculitis barbae –  Pustular psoriasis –  Pyoderma gangrenosum – Rosacea –  Subcorneal pustular dermatosis (Sneddon-Wilkinson) – Syphilis – Varicella –  Acanthosis nigricans –  Actinic keratosis – Angiosarcoma –  Basal cell carcinoma –  Candida infection – Cellulitis –  Cutaneous T-cell lymphoma –  Dermatofibrosarcoma protuberans – Dermatomyositis –  Diaper dermatitis – Eczema – Erythrasma –  Granuloma annulare, granuloma faciale – Ichthyosis –  Lichen (planus, sclerosus, simplex chronicus) –  Lupus erythematosus (discoid) –  Lyme disease – Melanoma – Morphea –  Necrobiosis lipoidica –  Nevus sebaceous –  Paget’s disease (mammary, extra-mammary) –  Pityriasis rosea, versicolor –  Pityriasis rubra pilaris – Psoriasis – Sarcoidosis –  Seborrheic dermatitis –  Sweet syndrome – Syphilis –  Tinea (corporis, pedis) – Vasculitis – Xanthelasma

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3  Dermatologic Concepts and Terminology Table 3.7 (continued) Tumoral (benign/malignant) – (Angio)lipoma – Acrochordon – Angioleiomyoma –  Atypical fibroxanthoma –  Basal cell carcinoma –  B-cell lymphoma – Dermatofibroma –  Dermatofibrosarcoma protuberans –  Hemangioma (infantile, congenital, etc.) –  Kaposi sarcoma – Keratoacanthoma – Liposarcoma –  Lymphocytoma cutis –  Malignant melanoma (variants) –  Merkel cell carcinoma – Metastasis –  Mycosis fungoides – Neurofibroma – Neuroma –  Nevi (melanocytic, congenital, acquired, etc.) –  Nevus sebaceous – Pilomatrixoma –  Pleomorphic dermal sarcoma – Pseudolymphoma –  Pyogenic granuloma –  Sebaceous adenoma –  Seborrheic keratosis –  Squamous cell carcinoma –  Syringocystadenoma papilliferum –  Verrucous carcinoma – Warts Vesicles/bullae Vesicles –  Acropustulosis of infancy –  Acute dermatitis (toxic, irritant) –  Bullous lichen planus –  Cutaneous candidiasis –  Darier disease –  Dermatitis herpetiformis (Duhring) –  Erythema multiforme –  Hand-foot-mouth disease –  Herpes simplex, zoster – Hidrocystoma –  Id reaction – Impetigo –  Incontinentia pigmenti –  Insect bites –  Linear IgA dermatosis – Mastocytosis –  Miliaria cristallina, rubra –  Pemphigus foliaceus – Pityriasis lichenoides et varioliformis acuta (PLEVA) –  Porphyria cutanea tarda – Scabies – Transient acantholytic dermatosis (Grover’s disease) – Varicella – Vasculitis Nodules and tumors

Cystic –  Branchial cleft cyst –  Bronchogenic cyst – Cutaneous metaplastic synovial cyst –  Cystic hygroma –  Dermoid cyst –  Digital mucous cyst –  Ear pit cyst –  Epidermoid cyst – Ganglion – Hidrocystoma – Mucocele –  Pilonidal cyst – Steatocystoma –  Thyroglossal duct cyst –  Trichilemmal cyst –  Vellus hair cyst

Nodular/inflammatory – Amyloidosis –  Calcinosis cutis – Carbuncle – Chondrodermatitis nodularis helices – Erythema induratum Bazin –  Erythema nodosum – Foreign body granuloma – Furuncle – Gout – Hidradenitis suppurativa –  Injection granuloma – Keloid – Lymphogranuloma venereum –  Osteoma cutis – Panniculitis –  Piezogenic nodules –  Polyarteritis nodosa –  Prurigo nodularis –  Rheumatoid nodule –  Scabies nodules – Sporotrichosis –  Tuberous xanthoma – Vasculitis

Bullae –  Bulla diabeticorum –  Bullous lichen planus –  Bullous pemphigoid – Burns – Cellulitis – Chilblains –  Cicatricial pemphigoid –  Contact dermatitis –  Epidermolysis bullosa acquisita –  Fixed drug eruption –  Friction blister –  Graft vs. host disease – Impetigo –  Linear IgA dermatosis –  Neonatal sucking blisters –  Pemphigoid gestationis –  Pemphigus vulgaris – Perniosis – Phytophotodermatitis – Pompholyx –  Staphylococcal scaled skin syndrome –  Toxic epidermal necrolysis

(continued)

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D. Crisan and M. Crisan

Table 3.7 (continued) Erosions/ulcers Ulcers – Aphthae –  Arterial ulcer –  Basal cell carcinoma – Chancroid – Decubitus –  Diabetic ulcer – Ecthyma –  Necrobiosis lipoidica –  Neuropathic ulcer –  Pressure ulcer –  Pyoderma gangrenosum –  Septic emboli –  Spider bites –  Squamous cell carcinoma –  Stasis ulcer –  Traumatic ulcer –  Trigeminal trophic syndrome –  Trophic ulcer –  Venous ulcer Hives – Angioedema – Dermographism – Urticaria –  Urticaria pigmentosa (mastocytosis) –  Urticaria vasculitis Fissure –  Chapping (hands, feet) – Eczema – Intertrigo – Perleche Atrophy – Aging – Dermatomyositis –  Discoid lupus erythematosus –  Lichen sclerosus et atrophicus – Morphea –  Necrobiosis lipoidica – Radiodermatitis – Striae –  Topical/intralesional steroids Scar – Acne – Burns –  Hidradenitis suppurativa – Keloid – Porphyria – Varicella

are listed in the table as having more than one morphological aspect. The topographic classification of some of the most common skin diseases, as seen in Fig. 3.5, is also meant as additional support for physicians

Erosions –  Bullous pemphigoid – Burns – Candidiasis –  Dermatophytic infections –  Eczematous diseases –  Epidermolysis bullosa –  Erythema multiforme –  Fixed drug eruption – Herpangina – Intertrigo –  Lichen planus –  Neuropathic excoriations –  Pemphigus vulgaris – Perleche – Syphilis –  Toxic epidermal necrolysis –  Vesiculobullous diseases

in discussing and ruling out differential diagnoses of certain conditions, which are known to appear with predilection in specific areas of our body (Fig. 3.5).

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3  Dermatologic Concepts and Terminology Fig. 3.4  Algorithm for diagnosing skin conditions according to their morphology and distribution (Modified from [4])

Skin lesion

Single

Patch Plane angioma Erythema chronicum migrans Postinflammatory hyperpigmentation

Papule Dermal nevus Basal cell carcinoma Skin tag Juvenile xanthogranuloma

Multiple

Localized

Patch Solar lentigines Vitiligo Vasculitis

Papules/nodules Nodule Skin cancer (basal cell carcinoma, squamous cell carcinoma, melanoma, atypical fibroxanthoma) Dermal nevus Mastocytoma

Plaque Lichen simplex chronicus Eczema Bowen disease Superficial spreading melanoma

Condylomata Syringoma Lichen planus Acne vulgaris Rosacea Sporotrichoid nodules Erythema nodosum

Plaques Psoriasis Mycosis fungoides Nummular eczema Tinea versicolor Pityriasis rosea Congenital nevi with satellites

Vesicular/bullous Fixed bullous drug erruption Suction blister Thermal burn

Ulcer Basal cell carcinoma Diabetic, venous, arterial ulcer Primary syphilitic affect Pyoderma gangrenosum

Nodular Satellite metastasis Segmental neurofibromatosis Kaposi’s sarcoma Dermatofibrosarcoma protuberans Hidradenitis suppurative

Generalized

Patch Viral exanthema Generalized drug eruption

Papules/nodules Psoriasis Lichen planus Secondary syphilis Neurofibromatosis Nodular scabies Prurigo nodularis

Plaques Psoriasis Mycosis fungoides Pityriasis rubra pilaris

Nodular Metastasis melanoma Lipomas Neurofibromatosis Steatocystoma multiplex

Vesicular/bullous Varicella Bullous disorders (bullous pemphigoid, pemphigus vulgaris)

Vesicular/bullous Herpes simplex Herpes zoster Fixed drug eruption

Pustulous Folliculitis barbae Impetigo Herpes zoster

Pustulous Pustular psoriasis Acute generalized exanthematic pustulosis (AGEP)

Hair and scalp

Oral mucosa

Fig. 3.5  Guide to dermatologic diagnosis by affected region [1, 3, 9]

Systemic disorders: pyoderma gangrenosum

Drugs/reactive: fixed drug eruption, erythema multiforme, drug-induced striae, AGEP, SDRIFE, papular urticaria, TEN, BIS Developmental anomalies/genetic disorders: Hailey-Hailey disease, pseudoacanthosis nigricans, acrokeratosis verruciformis

Developmental anomalies/genetic disorders: punctate keratoderma, epidermolysis bullosa, acrokeratosis verruciformis Systemic disorders: mycosis fungoides, Sezary syndrome, scleroderma, necrobiosis lipoidica, arteriosclerosis

Immunologic: vitiligo, lichen sclerosus et atrophicus, mucous membrane pemphigoid, pemphigus vulgaris

Inflammatory: seborrheic eczema, contact dermatitis, diaper dermatitis, lichen simplex, inverse psoriasis, lichen planus, hidradenitis suppurativa, acrodermatitis enteropatica, pityriasis rubra pilaris, Reiter's syndrome, Behcet's disease

Infections/infestations: herpes simplex and ouster, molluscum contagiosum, candidiasis, tinea cruris, syphilis, lymphogranuloma venereurn, condyloma, pediculosis, scabies, balanitis, chancroid, erythrasma, folliculitis, furunculosis, intertrigo, pediculosis pubis, impetigo, furuncle

Benign lesions and tumoral pathology: pearly penile papules, warts, bowenoid papulosis, acrochordon, angiokeratoma, lymphangioma, extramammary Paget disease, melanoma, squamous cell carcinoma, Bowen's disease

Genital area

Systemic disorders: mycosis fungoides, Sezary syndrome, sarcoidosis, urticaria pigmentosum, eruptive xanthoma, pyoderma gangrenosum, granuloma anulare

Developmental anomalies/genetic disorders: Darier's disease, neurofibromatosis, ichtyosis, tuberous sclerosis, xeroderrna pigmentosum

Drugs/reactive: exanthematic drug eruption, papular urticaria, fixed drug eruption, AGEP, DRESS, TEN, SJS

Immunologic: lupus erythematosus, pemphigus vulgaris, bullous pemphigoid, vitiligo, dermatitis herpetiformis, scleroderma, IgA linear dermatosis

Inflammatory: acne, acne conglobata, contact dermatitis, seborrheic dermatitis, eczema, folliculitis, pityriasis rosea, psoriasis, pityriasis lichenoides, pityriasis rubs pilaris, erythroderma, parapsoriasis en plaques

Infections/infestations: viral exanthema, pityriasis versicolor, herpes zoster, tinea corporis, secondary syphilis, leprosy, molluscum contagiosum, erythema migrans, Kaposi's sarcoma, varicella

Benign lesions and tumoral pathology: melanocytic nevi, lipomas, senile angiomas, seborrheic keratosis, epidermal cysts, basal cell carcinomas, squamous cell carcinoma, Bowen's disease, melanoma, skin metastasis

Torso

Systemic disorders: granulomatous cheilitis, Crohn's disease

Drugs/reactive: fixed drug eruption, erythema multiforme, TEN, 515

Immunologic: lupus erythematosus, pemphigus vulgaris, cicatricial pemphigoid

Inflammatory: contact dermatitis, lichen planus, leukoplakia, Behcet's syndrome, Reiter's syndrome, trauma

Infections: impetigo, herpes simplex and zoster, candidiasis, varicella zoster, coxackie virus, measels, oral hair leukoplakia, dental sinus, Kaposi sarcoma, syphilitic chancre

Benign lesions and tumoral pathology: venous lakes, warts, hemangioma, mucous cysts, lentigines, pyogenic granuloma, actinic cheilitis, squamous cell carcinoma, melanoma

Developmental anomalies: aplasia cuts congenita, dermoid cyst

Immunologic: cutaneous lupus erythematosus, pemphigus vulgaris, mucous membrane pemphigoid, dermatitis herpetiformis

Inflammatory: seborrheic dermatitis, contact dermatitis, psoriasis, acne keloidalis nuchae, pilar cyst, juvenile xantogranuloma, prurigo nodularis

Infections/infestations: folliculitis, tinea capitis, kerion, pediculosis capitis, herpes zoster, furuncle, carbuncle

Benign lesions and tumoral pathology: actinic keratosis, nevi (nevus sebaceus, congenital nevi, melanocytic nevi), hemangioma, seborrheic keratosis, basal cell carcinoma, squamous cell carcinoma, melanoma, merkel cell carcinoma, microcystic adnexaI carcinoma, angiosarcoma, skin metastasis

Alopecia: alopecia areata, anagen effluvium, androgenetic alpecia, discoid lupus erythematosus, frontal fibrosing alopecia, lichen planopilaris, syphilis, telogen effluvium, tinea capitis, traction alopecia, trichotillomania

Drugs/reactive: erythema multiforme, TEN, SJS

Immunologic: chilblain lupus, scleroderma, epidermolysis bullosa, vitiligo

Inflammatory: contact dermatitis, pompholyx, juvenile plantar dermatosis, psoriasis, lichen planus, infantile acropustulosis, pityriasis rubra pilaris, Reiter's syndrome, granuloma anulare

Infections/infestation: tinea pedum, secondary syphilis, insect bites, scabies, paronychia, cutaneous larva migrans, handfoot-mouth disease, pitted keratolysis, viral exanthema

Benign lesions and tumoral pathology: callus, clavus, warts, pyogenic granuloma, eccrine poroma, melanoma in situ, malignant melanoms, squamous cell carcinoma, cutaneous metastasis, Bowen's disease, Kaposis' s sarcoma

Feet

Systemic disorders: mycosis fungoides, sarcoidosis, porphiria cutanea tarda, scleroderma

Developmental anomalies/genetic disorders: accesory digits, aquired digital fibrokeratoma, epidermolysis bullosa

Drugs/reactive: fixed drug eruption, erythema muttiforrne, photosensitive drug reactions, radiation dermatitis, TEN, SJS

Immunologic: dermatomyositis, chilblain lupus, scleroderma, graft versus host disease, vitiligo, lupus erythematosus, CREST syndrome

Inflammatory: contact dermatitis, pompholyx, psoriasis, lichen planus, pityriasis rubra pilaris, granuloma anulare

Infections/infestations: warts, impetigo, herpes simplex, herpetic whitlow, tinea manum, paronychia, secondary syphilis, orf, erisipeloid, scabies, cutaneous larva migrans, hand-mouth-foot disease, scabies, viral exanthema

Benign lesions and tumoral pathology: solar lentigines, warts, callus, clavus, pyogenic granuloma, glomus tumor, myxoid cyst, actinic keratosis, Bowen's disease, squamous cell carcinoma, keratoacanthoma, merkel-cell carcinoma, melanoma, onychornatricoma, onychopapiloma

Hands

Developmental anomalies/genetic disorders: Hailey-Hailey disease, Neurofibromatosis (axillary freckling), LEOPARD syndrome, pseudoxynthoma elasticum

Drugs/reactive: SDRIFE, fixed drug eruption, AGEP, TEN, SJS

Immunologic: bullous pemphigoid, vitiligo, pemphigus vulgaris

Inflammatory: acanthosis nigricans, contact dermatitis, Fox-Fordyce disease, hidradenitis suppurativa, lichen simplex chronicus, miliaria

Infections: erythrasma, fungal and yeast infections, intertrigo, pediculosis corporis, scabies, trichomycosis axillaris, bullous impetigo

Benign lesions and tumoral pathology: ephelids, acrochordon, epidermal cyst, Bowen's disease, basal cell carcinoma, melanoma

Axilla

Systemic disorders: xanthelasma, Sweet's syndrome, sarcoidosis, myxedema, erythropoietic protoporphiria, systemic amyloidosis

Developmental anomalies/genetic disorders: dermoid cyst, pits, accesory tragus, thyroglossal duct cyst, bronchogenic cyst, Peutz-Jeghers syndrome, Osler-Weber-Rendu syndrome, tuberous sclerosis, pseudoxanthoma elasticum, xeroderma pigmentosum

Drugs/reactive: urticaria, angioedema, fixed drug eruption, photodrug reaction, DRESS, TEN, SJS

Immunologic: lupus erythematosus, dermatomyositis, scleroderma, CREST, bullous pemphigoid, pemphigus vulgaris, vitiligo

Inflammatory: acne, rosacea, seborrheic dermatitis, contact dermatitis, atopic eczema, lichen simplex, pityriasis alba, perioral dermatitis, psoriasis, cysts, rnelasm a, eosinophilic folliculitis

Infections/infestations: impetigo, erysipelas, herpes simplex and zoster, tinea faciei, warts, Kaposi's sarcoma, fifth disease, lymphocytoma cuts, leishmaniasis, lupus vulgaris, gram negative folliculitis

Benign lesions and tumoral pathology: acrochordon, angioma, dermatosis papulosa nigra, milia, dermal nevus, syringoma, nevus of Ota, port wine stain, blue nevus, nevus depigmentosus, solar lentigo, actinic keratosis, basal cell carcinoma, squamous cell carcinoma, merkel cell carcinoma, keratoacanthoma, lentigo maligna (melanoma), Bowen's disease, microcystic adnex carcinoma, atypical fibroxanthoma, pyogenic granuloma

Face/neck

66 D. Crisan and M. Crisan

3  Dermatologic Concepts and Terminology

 elevant Scoring Systems R in Dermatology Special scoring systems have been developed for different dermatologic conditions, which help us confirm the diagnosis, assess the disease severity, and hence establish the best therapeutic approach. Furthermore, scoring systems can also be used in monitoring therapeutic responses of various conditions to specific therapies, for instance, in clinical trials. Some relevant scoring systems and indexes useful for dermatological practice are listed below.

67 Table 3.9  International Hidradenitis Severity Score System (IHS4) IHS4 (points)= Number of nodules Number of abscesses Number of sinus tracts Mild HS Moderate HS Severe HS

Suppurativa

×1+ ×2+ ×4 ≤3 points 4–10 points ≥11 points

Table 3.10  Sonographic scoring of hidradenitis suppurativa (SOS-HS)

Grading Description I Single fluid collection and dermal changes affecting a single body segment without fistulous tracts II Two to four fluid collections or a single Hidradenitis Suppurativa fistulous tract with dermal changes affecting up to two body segments Hurley Score: It represents the first non-­ III Five or more fluid collections or two or more quantitative severity score classification routinely fistulous tracts with dermal changes or involvement of three or more body segments used in the clinical setting to categorize patients

according to the presence of scarring or sinus tracts into three categories; furthermore, the Hurley scoring system enables the selection of the appropriate treatment modality for patients: while stage 1 patients are candidates for medical therapy, stage 2 patients might qualify for local surgery, and stage 3 patients usually require wide surgical excision of the sinus tracts and extensive scarring [31–33] (Table 3.8). International Hidradenitis Suppurativa Severity Score System (IHS4): This new proposed clinical score, which allows the dynamic assessment of the severity of hidradenitis suppurativa, requiring the identification and counting of three different types of lesions: nodules, abscesses, and sinus tracts [34] (Table 3.9). Table 3.8  Hurley scoring system Stage 1 Solitary or few isolated abscesses without scarring or sinus tracts (fistulas, tunnels) Stage 2 Recurrent abscesses in more than one area with the beginning formation of sinus tracts (fistulas, tunnels) Stage 3 Multiple, widespread abscesses with interconnected sinus tracts (fistulas, tunnels), severe scarring, and continuous leaking

Sonographic scoring of hidradenitis suppurativa (SOS-HS): provides significant information regarding the subclinical involvement in hidradenitis suppurativa, which is clinically unavailable and not considered in the other scoring systems, often leading to an underestimation of the disease severity [35] (Table 3.10).

Malignant Melanoma Breslow index: refers to the measurement of the vertical depth of the melanoma (mm), from the granular layer to the area of the deepest penetration point of the tumor; the Breslow thickness represents a very important prognostic factor in patients with primary cutaneous melanoma, its accuracy having significant implications for staging and therapeutic management [36] (Table 3.11). Clark level: histological grading system, referring to the depth of penetration of the melanoma according to the anatomic level [37] (Table 3.12).

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Table 3.11  Breslow index for malignant melanoma; prognosis of 5- and 10-year survival according to the Breslow depth [36] Breslow thickness (mm) 2–4 >2–4 >4 >4

2,1–4 >4

Ulceration status Without ulceration With ulceration With or without ulceration Without ulceration With ulceration Without ulceration With ulceration Without ulceration With ulceration

Table 3.12  Clark level Level I

Confined to the epidermis, also called melanoma in situ Level II Invasion of the papillary dermis Level III-IV Invasion of the papillary and reticular dermis Level V Invasion into the subcutis

Psoriasis Psoriasis Area and Severity Index (PASI): It tool used for the assessment of disease severity in psoriasis patients, used to document clinical improvement under therapy or in clinical trials; for each body region, a representative area with psoriatic lesions is selected and the intensity of erythema, scaling, plaque thickness is assessed on a scale ranging from 0 to 4 according to the severity (parameter 1); in a next step, the percentage of psoriatic involvement needs to be calculated for each body segment (parameter 2), (0- >90% involvement, corresponding to a scale from 0 to 6); finally, the parameters are multiplied by a given correction factor (corresponding to the body surface area) and added up for the four main body regions (head, trunk, upper limbs, lower limbs) for the PASI score [3, 9] (Table 3.13). Nail Psoriasis Severity Index (NAPSI): This represents a simple, numeric tool for the severity assessment of nail psoriasis, commonly used either in clinical trials or to evaluate response to treatment of psoriatic nails; the nail plate is

5-Year survival (%) 99 99

10-Year survival (%) 98 96

96 93 94 86 90 82

92 88 88 81 83 75

Table 3.13  Psoriasis area and severity index (PASI) Upper Lower Head Trunk limbs limbs Severity of skin lesions: 0—none, 1—slight, 2— moderate, 3—severe, 4—very severe Erythema 0–4 0–4 0–4 0–4 Induration 0–4 0–4 0–4 0–4 Scaling 0–4 0–4 0–4 0–4 Total score (A) Sum Sum Sum Sum of of of of above above above above Extent of involvement: 0—None, 1— 4  months after injection). The early complications can be subdivided into immediate (first 24–48  h) and intermediate (first 48  h to 4 months) complications.

Early Complications Immediate Complications Occlusion is the most severe complication and is typically evident in the first 24–48 h. One of

a

the most terrible complications is blindness, which is due to the occlusion of the retinal artery. This is secondary to the injection of the glabellar region that contains the supraorbital and supratrochlear arteries. The mechanism is the retrograde flow due to the injection of filler in the supraorbital or supratrochlear arteries and an anterograde flow with the filler into the central retinal artery, which can generate the occlusion of the central retinal artery [6, 7, 34–44]. Brain infarctions are also severe complications, and this can be done due to communications of the dorsal nasal arteries, the anterior and posterior ethmoidal arteries, and the ophthalmic arteries [6, 7, 34–44]. Cyanosis, edema, erythema, livedo reticularis, and epidermal necrosis can occur due to the occlusion of main and subcutaneous vessels in the injection region(s) (Fig.  22.2) [6, 7, 34–44].

b

Fig. 22.2  Upper lip ischemia after injection of hyaluronic acid. (a, b). Clinical images. (c, d). Ultrasound (c and e, grayscale; d, color Doppler; c and d at 24 MHz and e at 70 MHz; left border of the upper lip; c and e, before and d, 48  h after hyaluronidase). Notice the thickening and hypoechogenicity with loss of definition of the borders of the orbicularis oris muscle before hyaluronidase

and the recovery of the definition and echogenicity of the layers after hyaluronidase. On color Doppler, there is a reactive dermal and hypodermal hypervascularity due to occluded small hypodermal vessels (e, between markers). After hyaluronidase, the normal flow is recovered. The labial artery presented a normal flow before and after hyaluronidase injection

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c

d

e

Fig. 22.2 (continued)

Intermediate Complications Edema due to the inflammation of the subcutaneous fat, lymphatic congestion, and water retention due to the hydrophilic nature of some fillers can be found [6, 7, 34, 35, 37–39, 41–44]. Overfilling can produce palpable lumps and bumps in the injection region(s) (Fig. 22.3) [6, 7, 34, 35, 37–39, 41–44]. Management of Early Complications In cases presenting complications that have been injected with hyaluronic acid, hyaluronidase is usually the treatment. Ultrasound allows performing a percutaneous injection of hyaluronidase, which punctures directly in the occlusion or the overfilled region. In the rest of

the fillers, the management is more complex because many of them are not degradable; therefore, an extraction or dilution of the filler may be evaluated. Nevertheless, it has been reported that the ultrasound-guided injections of hyaluronidase allow us to use a lower dose (35–50 iu) in comparison with the high dose (500 iu>) usually used in non-guided procedures [13, 16–18, 23–26, 32, 38, 45].

Late Complications The complications that appear after 4 months of injection are the most frequent. Commonly, these are secondary to an autoimmune reaction of the host to the filler.

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dermis and hypodermis. In cases injected with hyaluronic acid, floating echoes may be found within the deposits [18, 24, 25, 28]. Granulomatous Reaction This appears on ultrasound as hypoechoic bands, tissue, nodules, or pseudonodules surrounding the deposits. Sometimes, this granulomatous reaction generates a pseudocapsule around the filler (Fig. 22.4) [15, 18, 24, 25].

b

Fig. 22.3  Overfilling with hyaluronic acid in the tip of the nose. (a) Clinical images show a bulging tip of the nose. (b) Ultrasound (longitudinal view) shows a 1.93 cm long deposit of hyaluronic acid (between markers) in the hypodermis

Among these late adverse reactions are chronic inflammation, granulomatous reactions, sarcoid reactions, panniculitis, morphea-like reactions, lumps and bumps, and inflammation of the lacrimal, parotid, and submandibular glands [9, 15, 16, 18, 22, 25, 26, 28, 32, 46]. Chronic Inflammation This is an inflammatory reaction of the surrounding dermis and hypodermis. There is thickening and/or echogenicity alteration of the dermis and hypodermis surrounding the deposits. The dermis becomes hypoechoic, and the hypodermis turns hyperechoic. Not infrequently, there is regional hypervascularity with slow-flow vessels of the

Sarcoid Reaction This large granulomatous reaction extends beyond the injection sites and could be present in scars of previous procedures (Fig. 22.5) [15, 18, 24, 25]. Panniculitis This is the inflammation of the hypodermis and may present a lobular or mixed pattern (lobular-­ septal) type. On ultrasound, hyperechogenicity of the hypodermis presents a diffuse pattern (lobular presentation) or is associated with hypoechoic thickening of the septa (mixed pattern) in late cases. A variable degree of hypodermal hypervascularity may be found [18, 24, 28, 46]. You can look for more information and images about panniculitis in the chapter on inflammatory cutaneous diseases (Chap. 18). Morphea and Morphea-Like Reactions Clinically, they present as erythematous, violaceous, or hyperpigmented regions that correlate with the location of the deposits and are frequently associated with regional dermal and hypodermal inflammatory signs. On ultrasound, they present the pattern already reported for morphea. The ultrasonographic morphea signs are decreased echogenicity of the dermis, increased echogenicity of the hypodermis, loss of definition of the dermal-hypodermal border, and dermal and/or hypodermal hypervascularity. The most sensitive signs for detecting the activity of morphea are increased hypodermal echogenicity and dermal and/or hypodermal hypervascularity (Fig. 22.6) [18, 24, 28].

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b

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Fig. 22.4  Granulomatous reaction after injection with hyaluronic acid 4  months ago. (a) Clinical image. The patient presented a palpable lump on the left side of the chin. (b, c) Ultrasound (b, grayscale longitudinal view; c, color Doppler, transverse view; left side of the chin). There is ill-defined hypoechoic granulomatous tissue (*)

a

in the deep hypodermis and depressor anguli oris muscle (dm). Notice a remnant of hyaluronic acid (ha) besides the granulomatous reaction. On color Doppler, there is hypervascularity in the periphery of the granulomatous reaction

b

c

Fig. 22.5  Sarcoid reaction after injection with hyaluronic acid 1 year ago. (a) Clinical image of a patient with induration and erythema of the cheeks. (b, c) Ultrasound transverse views (b, grayscale and c, color Doppler; right cheek) present hypoechoic tissue with lobulated borders

and pseudonodules in the hypodermis musculoaponeurotic layers (between markers). On color Doppler, there is increased vascularity in the periphery of the hypoechoic tissue

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c

Fig. 22.6  Active morphea after the injection of two types of fillers in the face. (a) Clinical photograph. Patient presented induration of the frontal region. (b, c) Ultrasound images (transverse views; b, at 46  MHz, right nasofold line; (c) at 24 MHz, frontal glabellar region). In b, there are two types of fillers: hyaluronic acid (ha) and calcium

hydroxyapatite (Ca H) in the hypodermis of the right nasofold line. In the glabellar region (c), there is decreased echogenicity of the dermis, increased echogenicity of the hypodermis, and loss of definition of the dermal-­ hypodermal border (arrow)

Lumps and Bumps These can be caused by superficial deposits of fillers or inflammatory and/or granulomatous reactions surrounding the deposits. The presence of multiple lumps and bumps in the face may cause a facial clinical disfiguration. On ultrasound, the lumps and bumps can be caused by filler deposits and/or an inflammatory reaction such as secondary panniculitis or a granulomatous reaction [18, 24, 28].

ducts. On color Doppler, there is a variable degree of hypervascularity of the glands (Fig. 22.7). It is thought that this subacute autoimmune reaction could be categorized as part of the ASIA syndrome (autoimmune inflammatory syndrome induced by adjuvant syndrome) caused by exogenous materials. This is relevant because the glands’ inflammation can generate dry eyes (xerophthalmia) or dry mouth (xerostomia). Dry eyes have been keratoconjunctivitis, chronic conjunctivitis, sterile keratolysis, nonhealing corneal ulcers, uveitis, scleritis, retinal, vasculitis, and optic neuritis. Dry mouth can cause difficulties in swallowing, chewing, speaking, burning sensation, halitosis, altered taste, dryness of the buccal mucosa, glossitis, cracked and peeled lips, oral candidiasis, and dental caries [47].

Inflammation of the Lacrimal, Parotid, and Submandibular Glands This has been reported in cases with all types of cosmetic fillers and generates hypoechogenicity or heterogeneous echogenicity of the glands. Commonly, the size of the glands remains normal, and there is no dilation of the glandular

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a

b

c

d

Fig. 22.7  Ultrasonographic inflammatory signs of the glands after injection with hyaluronic acid ultrasound. (a) Hyaluronic acid deposits (*) in the hypodermis of the cheek. (b) Lacrimal gland, (c) parotid gland, and (d) submandibular gland demonstrate hypoechogenicity in all the

glands with hypoechoic pseudonodules in the parotid and submandibular gland. These findings were bilateral, although there is only one gland per side in the figures. On color Doppler, there is mild hypervascularity of the lacrimal gland

Nonsurgical Aesthetic Procedures

Mesotherapy can cause inflammation of the dermis and/or hypodermis, which is more frequent in the hypodermis generating lobular or mixed types of panniculitis, and sometimes a granulomatous reaction. Thus, decreased echogenicity of the dermis and increased echogenicity of the hypodermis can be detected. In cases with granulomatous reactions, hypoechoic tissue, nodules, or pseudonodules are seen. Some cases may present regional lymphedema signs with thickening of the epidermis, dermis, and hypodermis, besides the echogenicity alterations in the same layers. Rarely, fluid collections or abscesses may be detected in the injection sites. On color Doppler, there is a variable degree of hypervascularity in the affected layers (Figs. 22.8 and 22.9) [24, 25].

The role of ultrasound in nonsurgical aesthetic procedures has been directed to detect complications of the procedures.

Mesotherapy This is the use of lipolytic or cosmeceutical agents in aesthetics. It can be used for treating cellulitis or alopecia and for rejuvenation purposes. Examples of mesotherapy agents are pentoxifylline, carnitine, coumarin, hyaluronidase/colla genase, calcium pyruvate, aminophylline/caffeine, artichoke, melilotus or ginkgo biloba, multivitamins, and T3/T4 [8, 48–50].

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c

Fig. 22.8 Mixed panniculitis post-mesotherapy. (a) Clinical image with a lesion in the lower quadrant after treatment with a lipolytic agent. (b, c) Ultrasound (b, grayscale and c, color Doppler ultrasound) presented

a

b

c

Fig. 22.9  Granulomatous reaction after mesotherapy. (a) Clinical photograph shows tiny erythematous bumps in the frontal region. (b, c) Grayscale ultrasound (b, at 24 MHz and c, at 70 MHz) presents hypoechoic granulomatous tissue (*), which is located in the dermis in b and both in the dermis and hypodermis in c

mixed hypodermal panniculitis with thickening and increased echogenicity of the subcutis (*) and some hypoechoic thickening of the septa. On color Doppler, there is a slight hypervascularity in the lesion’s periphery

Cryolipolysis Also called CoolSculpting, fat freezing, and lipofreezing, this procedure [24, 25] uses a device that exposes the fatty tissue to very low temperatures (usually −2 to −10  °C). The aim is to decrease the fatty tissue [51, 52]. On ultrasound, there is hyperechogenicity and/or heterogenicity of the hypodermis and sometimes anechoic round- or oval-shaped structures suggestive of fat liquefaction or necrosis. On color Doppler, the degree of vascularity will depend on the amount of inflammation (Fig. 22.10) [24, 25].

Radiofrequency This procedure aims to heat the superficial layers, ideally the dermis, and is used for managing skin laxity [53]. On ultrasound, there is dermal and hypodermal thickening, hypoechogenicity of the dermis, and hyperechogenicity of the hypodermis. Sometimes, there is also thickening of the hypodermal septa. On color Doppler, the vascularity is variable and can go from hypovascular to hypervascular (Fig. 22.11) [24, 25].

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b

c

Fig. 22.10  Lobular panniculitis after cryolipolysis. (a) Clinical image of the inner arm that presented a palpable induration. (b, c) Ultrasound (b, grayscale and c, color

Doppler) shows hyperechoic and heterogeneous areas in the hypodermis (*, between markers). On color Doppler, a few vessels are noticed in the periphery of these areas

Autologous Fat Grafting

Tensor Threads

It is also called autologous fat transfer, lipotransfer, liposculpting, and lipofilling. And it relies on the injection of fat for soft-tissue augmentation. It is usually nonsurgical, but it can be added to a surgical procedure [38, 54–56]. On ultrasound, fat grafts appear as round- or oval-shaped hypoechoic nodules that present fibrous hyperechoic septa. These structures disrupt the normal architecture of the tissues and do not follow the axes of the cutaneous layers. They are usually located in the hypodermis, but they may also be found within the facial muscles, particularly in the orbicularis oculi muscles of the eyelid or the orbicularis oris of the lips. On color Doppler, fat grafts are hypovascular (Fig. 22.12) [24, 25, 38].

These are called Russian, facial, and wire threads and are usually used to perform noninvasive lifting. These can be divided into absorbable or nonabsorbable, and barbed and non-barbed. Nowadays, the most used are the absorbable non-­ barbed types such as those composed of polydioxanone (PDO tensor threads), a material also used in the sutures [57–61]. On ultrasound, these appear as bilaminar or trilaminar hyperechoic structures that sometimes generate a slight posterior acoustic shadowing artifact. They are commonly inserted in the hypodermis; however, they may sometimes be found in the dermis, which usually causes complications. Besides treating the sagging of the skin, the objective is to produce collagen and fibrosis;

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a

b

b

c

Fig. 22.11  Mixed panniculitis after radiofrequency. (a) Clinical image shows erythema and induration in the inner part of the arm. (b, c) Ultrasound (b, grayscale and c, color Doppler; transverse views) demonstrates increased echogenicity of the hypodermis with hypoechoic thickening of the superficial septa, besides thickening and decreased echogenicity of the dermis. On color Doppler, there is increased dermal and hypodermal vascularity due to inflammation

however, absorbable threads become rapidly fragmented over time, frequently in 2 or 3 months, and the tension is lost. When threads are fragmented and located near or within the dermis, granulomatous inflammatory hypoechoic tissue can be found surrounding the fragments. On color Doppler, the vascularity is variable and will depend on the amount of inflammation in the periphery of the threads (Fig. 22.13) [26].

Fig. 22.12  Fat grafts. Ultrasound images (grayscale; a, longitudinal lower eyelid and upper cheek; b, transverse view lower eyelid) present oval-shaped hypoechoic structures (*, between markers) that distort the normal anatomy of the region. In a, the fat graft is located in the hypodermis, and in b, the fat graft is on the surface of the lower fat pad of the orbit

Implants These are synthetic structures that are used for restoring volume and improving contour. There are several types of implants, and they could be inserted in multiple parts of the body, including the nose, cheek, chin, gluteal region, or calf. Implants can be composed of pure silicone gel or saline, porous high-density polyethylene, autologous cartilage, fat, or bone [24–26, 62]. On ultrasound, these implants appear different according to the material. Silicone implants show as well-defined oval-­ shaped anechoic structures. In their periphery, they present a monolaminar, bilaminar, or trilaminar outer layer. These implants can be ruptured, and there are ultrasonographic signs that suggest intracapsular or extracapsular rupture. Intracapsular rupture is characterized by the collapse of the implant, internal echoes, wavy lines within the implant, also called the “stepladder sign,” and discontinuity of the margin,

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a

b

c Fig. 22.14 Ultrasonographic patterns of common implants used in aesthetics

ing to the level of inflammation (Fig. 22.14) [24, 25]. Fig. 22.13 Tensor threads  complication (a) Clinical image shows erythema, retraction, and bumps in the right cheek after 2  months of polydioxanone (PDO) tensor thread procedure. (b, c) Ultrasound images (grayscale, transverse views; b, grayscale and c, color Doppler) show two hyperechoic bilaminar structures in the dermis with hypoechoic inflammatory and granulomatous tissue. Notice the hypervascularity surrounding the tensor threads (c)

Extracapsular rupture features are hyperechoic deposits with the “snowstorm” sign, a diffuse posterior acoustic reverberance in the periphery of the implant. Polyethylene implants appear as well-defined, hyperechoic bands. Cartilage implants show as well-defined, hypoechoic bands. On color Doppler, there is a variable degree of vascularity in the periphery of the implant accord-

Surgical Aesthetic Procedures Liposuction This is the surgical removal of fatty hypodermal tissue through vacuum suction [63]. On ultrasound, this procedure usually shows inflammatory signs with increased echogenicity of the hypodermis and areas that lack fatty lobules. It is not uncommon to find hypoechoic hypodermal serohematic fluid collections and anechoic pseudocystic structures suggestive of fat necrosis in the liposuction areas. At later stages, laminar hyperechoic hypodermal bands suggestive of fibrous tissue can be detected in the liposuction regions (Fig.  22.15) [24, 25].

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Fig. 22.15  Liposuction and abdominoplasty changes (a) Clinical image. (b, c) Ultrasound (transverse views; b, grayscale at the right flank and c, color Doppler at the left flank). (b) shows hyperechogenicity of the hypodermis and oval-shaped anechoic areas that correspond to fat necrosis (arrowheads). (c) presents heterogeneous dermal

and hypodermal echogenicity that predominates in the hypodermis  (*) with some anechoic zones (arrowheads) compatible with fat necrosis (b), and parts without fatty lobules  (c). There is also a mild hypodermal hypervascularity (c)

Abdominoplasty

with remnants and fragments of sutures [24, 25]. Hypertrophic scars or keloids are also ­complications of abdominoplasty and appear as a hypoechoic dermal thickening in the scar region that extends beyond the original borders of the scar in keloids. Ultrasound can support the detection of activity in keloids [24, 65].

Also called a tummy tuck, it relies on the surgical removal of the excess of skin and hypodermal fat and the tightening of the anterior rectus muscles. Abdominoplasty [64] is commonly performed in concomitance with liposuction and includes the creation of a neoumbilicus and a wide suprapubic scar that connects the lower abdomen and both hips. On ultrasound, there is hyperechogenicity of the hypodermis, and some cases may present hypoechogenicity and increased thickness of the dermis (Fig. 22.15). Frequently, there is thickening and hypoechogenicity of the sheath of the anterior rectus muscles. Among complications of abdominoplasty are anechoic or hypoechoic serohematomas and less commonly fistulous tracts usually associated

Blepharoplasty It considers the removal of the sagging skin and fat excess in the eyelid regions. In the upper eyelid, the incision goes through the eyelid crease and the upper orbitary septum and removes the excess of skin, the protruding intraorbital extraconal fat, and, if necessary, the corresponding section of the orbicularis oculi muscle [66].

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Fig. 22.16 Blepharoplasty  complication (a) Clinical image with erythema and edema of the upper eyelids 1  month after blepharoplasty. (b, c) Ultrasound images (grayscale transverse views; b at 24  MHz and c at 70  MHz) demonstrate hypoechoic inflammatory and granulomatous tissue (*) in the dermis, orbicularis muscle, and surface of the underlying intraorbital fat pad in

the lateral part of the right upper eyelid. In c, notice the bilaminar hyperechoic fragments corresponding to remaining sutures in the same region. (d) Color Doppler of the same region demonstrates hypervascularity in the dermis, orbicularis muscle and the surface of the intraprbital fat pad due to inflammation

In the lower eyelid, the approach is transconjunctival and through the lower orbitary septum and removes the excess of intraorbital extraconal fat. In cases with hypertrophy of the lower orbicularis oculi muscle, the incision is performed through the skin [66]. On ultrasound, there is decreased dermal and muscular echogenicity and hyperechogenicity of the fat pad. Commonly, there are laminar remnants of anechoic or hypoechoic serohematic fluid collections in the upper and lower intraorbitary extraconal fat pads. In some cases, hyperechoic bilaminar fragments that correspond to sutures may be found. In the periphery of these fragments, there is hypoechoic inflammatory and granulomatous tissue (Fig. 22.16). At late stages, it is possible to detect thinning of the orbicularis muscle and sometimes hypoechoic nodules that correspond to granulomas.

On color Doppler, there is a variable degree of hypervascularity in the tissues that can go from hypo- to hypervascular [24–26].

Rhinoplasty The surgical procedure implies the change of the shape of the nose to improve the aesthetic appearance and/or function [67]. Changes in the shape of the nose can also be performed by the injection of fillers, which is called rhinomodulation or filler rhinoplasty [67]. On ultrasound, the findings will depend on the technique used in the procedure, but for example, there could be hypoechoic inflammatory or granulomatous hypodermal or subaponeurotic tissue, hyperechoic linear fragments of sutures, hypoechoic bands suggestive of cartilage implants  (cartilage  grafts), irregularities of the

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Fig. 22.17  Rhinoplasty complication (a) Clinical image of a patient with an erythematous bump in the nasal dorsum 4 months after rhinoplasty. (b, c) Ultrasound images (b, grayscale longitudinal view; c, color Doppler, transverse view of the nasal dorsum) show dermal extrusion (arrow) of one of the cartilage implants (*, between markers) located in the dorsum of the nose. On color Doppler, there is dermal and hypodermal hypervascularity in the periphery of the cartilage implants

nasal bones, and lack of parts of the upper nasal cartilages. Other types of implants can also be detected, such as hyperechoic bands suggestive of polyethylene implants. In some cases, open rhinoplasty and filler rhinoplasty (rhinomodulation) are combined (Fig. 22.17) [24, 26].

References 1. Angelos PC, Been MJ, Toriumi DM.  Contemporary review of rhinoplasty. Arch Facial Plast Surg. 2012;14(4):238–47. https://doi.org/10.1001/ archfacial.2012.577. 2. Attenello NH, Maas CS.  Injectable fillers: review of material and properties. Facial Plast Surg. 2015;31(1):29–34. https://doi. org/10.1055/s-­0035-­1544924. 3. Bass LS. Injectable filler techniques for facial rejuvenation, volumization, and augmentation. Facial Plast Surg Clin North Am. 2015;23(4):479–88. https://doi. org/10.1016/j.fsc.2015.07.004. 4. Chia CT, Neinstein RM, Theodorou SJ.  Evidence-­ based medicine: liposuction. Plast Reconstr Surg. 2017;139(1):267e–74e. https://doi.org/10.1097/ prs.0000000000002859. 5. Garvey PB, Ricciardelli EJ, Gampper T.  Outcomes in threadlift for facial rejuvenation. Ann Plast Surg. 2009;62(5):482–5. https://doi.org/10.1097/ SAP.0b013e31818c18ed. 6. Ginat DT, Schatz CJ.  Imaging features of midface injectable fillers and associated complications. AJNR Am J Neuroradiol. 2013;34(8):1488–95. https://doi. org/10.3174/ajnr.A3161. 7. Glogau RG. Fillers: from the past to the future. Semin Cutan Med Surg. 2012;31(2):78–87. https://doi. org/10.1016/j.sder.2012.03.004. 8. Lee JC, Daniels MA, Roth MZ.  Mesotherapy, microneedling, and chemical peels. Clin Plast Surg. 2016;43(3):583–95. https://doi.org/10.1016/j. cps.2016.03.004. 9. Benito-Ruiz J, de Cabo F.  Ultrasonography: a useful tool for plastic surgeons. Aesthet Plast Surg. 2014;38(3):561–71. https://doi.org/10.1007/ s00266-­014-­0300-­z. 10. Faundez E, Vega N, Vera E, Vega P, Sepulveda D, Wortsman X.  Clinical and color Doppler ultrasound evaluation of polyacrylamide injection in HIV patients with severe facial lipoatrophy secondary to antiretroviral therapy. Skin Res Technol. 2017;23(2):243–8. https://doi.org/10.1111/srt.12329. 11. Grippaudo FR, Mattei M.  High-frequency sonography of temporary and permanent dermal fillers. Skin Res Technol. 2010;16(3):265–9. https://doi. org/10.1111/j.1600-­0846.2010.00428.x. 12. Grippaudo FR, Mattei M.  The utility of high-­ frequency ultrasound in dermal filler evaluation. Ann Plast Surg. 2011;67(5):469–73. https://doi. org/10.1097/SAP.0b013e318203ebf6. 13. Kwon HJ, Kim BJ, Ko EJ, Choi SY.  The utility of color Doppler ultrasound to explore vascular complications after filler injection. Dermatol Surg. 2017;43(12):1508–10. https://doi.org/10.1097/ dss.0000000000001173.

22  Ultrasound in Aesthetics 14. Lee GSK.  Use of handheld ultrasound Doppler to prevent complications from intra-arterial injection of dermal fillers: clinical experience. J Cosmet Dermatol. 2019; https://doi.org/10.1111/ jocd.12870. 15. Mlosek RK, Skrzypek E, Skrzypek DM, Malinowska S.  High-frequency ultrasound-based differentiation between nodular dermal filler deposits and foreign body granulomas. Skin Res Technol. 2018;24(3):417– 22. https://doi.org/10.1111/srt.12444. 16. Schelke LW, Van Den Elzen HJ, Erkamp PP, Neumann HA.  Use of ultrasound to provide overall information on facial fillers and surrounding tissue. Dermatol Surg. 2010;36(Suppl 3):1843–51. https:// doi.org/10.1111/j.1524-­4725.2010.01740.x. 17. Schelke LW, Velthuis P, Kadouch J, Swift A.  Early ultrasound for diagnosis and treatment of vascular adverse events with hyaluronic acid fillers. J Am Acad Dermatol. 2019; https://doi.org/10.1016/j. jaad.2019.07.032. 18. Wortsman X.  Identification and complications of cosmetic fillers: sonography first. J Ultrasound Med. 2015;34(7):1163–72. https://doi.org/10.7863/ ultra.34.7.1163. 19. Wortsman X, Quezada N.  Ultrasound morphology of Polycaprolactone filler. J Ultrasound Med. 2017;36(12):2611–5. https://doi.org/10.1002/ jum.14327. 20. Wortsman X, Wortsman J.  Polyacrylamide fillers on skin ultrasound. J Eur Acad Dermatol Venereol. 2012;26(5):660–1. https://doi. org/10.1111/j.1468-­3083.2011.04111.x. 21. Wortsman X, Wortsman J, Orlandi C, Cardenas G, Sazunic I, Jemec GB. Ultrasound detection and identification of cosmetic fillers in the skin. J Eur Acad Dermatol Venereol. 2012;26(3):292–301. https://doi. org/10.1111/j.1468-­3083.2011.04047.x. 22. Young SR, Bolton PA, Downie J.  Use of high-­ frequency ultrasound in the assessment of injectable dermal fillers. Skin Res Technol. 2008;14(3):320–3. https://doi.org/10.1111/j.1600-­0846.2008.00297.x. 23. Quezada-Gaón N, Wortsman X.  Ultrasound-guided hyaluronidase injection in cosmetic complications. J Eur Acad Dermatol Venereol. 2016;30(10):e39–40. https://doi.org/10.1111/jdv.13286. 24. Wortsman X.  Atlas of dermatologic ultrasound. 1st ed. Berlin: Springer International Publishing; 2018. p. 367. 25. Wortsman X, Jemec GBE.  Dermatologic ultrasound with clinical and histologic correlations. 1st ed. New York: Springer-Verlag; 2013. 26. Wortsman X, Wortsman J.  Sonographic outcomes of cosmetic procedures. AJR Am J Roentgenol. 2011;197(5):W910–8. https://doi.org/10.2214/ AJR.11.6719. 27. Wortsman X, Alfageme F, Roustan G, et  al. Guidelines for performing dermatologic ultrasound examinations by the DERMUS group. J Ultrasound

431 Med. 2016;35(3):577–80. https://doi.org/10.7863/ ultra.15.06046. 28. Wortsman X. Common applications of dermatologic sonography. J Ultrasound Med. 2012;31(1):97–111. https://doi.org/10.7863/jum.2012.31.1.97. 29. Alfageme F, Wortsman X, Catalano O, et al. European Federation of Societies for ultrasound in medicine and biology (EFSUMB) position statement on dermatologic ultrasound. Ultraschall Med. 2021;42(1):39–47. Stellungnahme der European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) zu Dermatologischem Ultraschall. https://doi. org/10.1055/a-­1161-­8872. 30. Wortsman X, Alfageme F, Roustan G, et al. Proposal for an assessment training program in dermatologic ultrasound by the DERMUS group. J Ultrasound Med. 2016;35(11):2305–9. https://doi.org/10.7863/ ultra.15.10068. 31. Wortsman X.  Ultrasound in dermatology: why, how, and when? Semin Ultrasound CT MR. 2013;34(3):177–95. https://doi.org/10.1053/j. sult.2012.10.001. 32. Wortsman X. Sonography of dermatologic emergencies. J Ultrasound Med. 2017;36(9):1905–14. https:// doi.org/10.1002/jum.14211. 33. Schelke LW, Cassuto D, Velthuis P, Wortsman X.  Nomenclature proposal for the sonographic description and reporting of soft tissue fillers. J Cosmet Dermatol. 2020;19(2):282–8. https://doi. org/10.1111/jocd.13127. 34. Beauvais D, Ferneini EM.  Complications and litigation associated with injectable facial fillers: a cross-sectional study. J Oral Maxillofac Surg. 2020;78(1):133–40. https://doi.org/10.1016/j. joms.2019.08.003. 35. Dayan SH, Ellis DA, Moran ML.  Facial fillers: discussion and debate. Facial Plast Surg Clin North Am. 2012;20(3):245–64. https://doi.org/10.1016/j. fsc.2012.05.004. 36. Doerfler L, Hanke CW. Arterial occlusion and necrosis following hyaluronic acid injection and a review of the literature. J Drugs Dermatol. 2019;18(6):587. 37. Fitzgerald R, Bertucci V, Sykes JM, Duplechain JK.  Adverse reactions to injectable fillers. Facial Plast Surg. 2016;32(5):532–55. https://doi. org/10.1055/s-­0036-­1592340. 38. Kadouch J, Schelke LW, Swift A.  Ultrasound to improve the safety and efficacy of Lipofilling of the temples. Aesthet Surg J. 2021;41(5):603–12. https:// doi.org/10.1093/asj/sjaa066. 39. Mack WP. Complications in periocular rejuvenation. Facial Plast Surg Clin North Am. 2010;18(3):435–56. https://doi.org/10.1016/j.fsc.2010.05.002. 40. Roberts SA, Arthurs BP. Severe visual loss and orbital infarction following periorbital aesthetic poly-(L)-lactic acid (PLLA) injection. Ophthalmic Plast Reconstr Surg. 2012;28(3):e68–70. https://doi.org/10.1097/ IOP.0b013e3182288e4d.

432 41. Vanaman M, Fabi SG, Carruthers J.  Complications in the cosmetic dermatology patient: a review and our experience (part 2). Dermatol Surg. 2016;42(1):12–20. https://doi.org/10.1097/01. Dss.0000479796.34703.94. 42. Vanaman M, Fabi SG, Carruthers J. Complications in the cosmetic dermatology patient: a review and our experience (part 1). Dermatol Surg. 2016;42(1):1–11. https://doi.org/10.1097/dss.0000000000000569. 43. Woodward J, Khan T, Martin J.  Facial filler complications. Facial Plast Surg Clin North Am. 2015;23(4):447–58. https://doi.org/10.1016/j. fsc.2015.07.006. 44. Yahyavi-Firouz-Abadi N, Menias CO, Bhalla S, Siegel C, Gayer G, Katz DS.  Imaging of cosmetic plastic procedures and implants in the body and their potential complications. AJR Am J Roentgenol. 2015;204(4):707–15. https://doi.org/10.2214/ ajr.14.13516. 45. Menis D, Castellanos-González M, Llamas-Martín R, Vanaclocha SF. The utility of skin ultrasound for the diagnosis of complications of tissue filler materials. Actas Dermosifiliogr. 2014;105(8):797–8. https://doi. org/10.1016/j.ad.2013.11.012. 46. Pérez-Pérez L, García-Gavín J, Wortsman X, Santos-­ Briz Á. Delayed adverse subcutaneous reaction to a new family of hyaluronic acid dermal fillers with clinical, ultrasound, and histologic correlation. Dermatol Surg. 2017;43(4):605–8. https://doi.org/10.1097/ dss.0000000000000945. 47. Wortsman X, Moll-Manzur C, Ramírez-Cornejo C, et  al. Ultrasonographic subclinical signs of inflammation of the lacrimal, parotid, and submandibular glands in users of cosmetic fillers. J Ultrasound Med. 2021;40(11):2377–89. https://doi.org/10.1002/ jum.15621. 48. El-Domyati M, El-Ammawi TS, Moawad O, et  al. Efficacy of mesotherapy in facial rejuvenation: a histological and immunohistochemical evaluation. Int J Dermatol. 2012;51(8):913–9. https://doi. org/10.1111/j.1365-­4632.2011.05184.x. 49. Tan J, Rao B.  Mesotherapy-induced panniculitis treated with dapsone: case report and review of reported adverse effects of mesotherapy. J Cutan Med Surg. 2006;10(2):92–5. https://doi. org/10.2310/7750.2006.00013. 50. Tedeschi A, Lacarrubba F, Micali G.  Mesotherapy with an intradermal hyaluronic acid formulation for skin rejuvenation: an Intrapatient, placebo-controlled, long-term trial using high-frequency ultrasound. Aesthet Plast Surg. 2015;39(1):129–33. https://doi. org/10.1007/s00266-­014-­0432-­1. 51. Ingargiola MJ, Motakef S, Chung MT, Vasconez HC, Sasaki GH. Cryolipolysis for fat reduction and body contouring: safety and efficacy of current treatment paradigms. Plast Reconstr Surg. 2015;135(6):1581– 90. https://doi.org/10.1097/prs.0000000000001236. 52. Krueger N, Mai SV, Luebberding S, Sadick NS.  Cryolipolysis for noninvasive body contour-

X. Wortsman ing: clinical efficacy and patient satisfaction. Clin Cosmet Investig Dermatol. 2014;7:201–5. https://doi. org/10.2147/ccid.S44371. 53. Beasley KL, Weiss RA. Radiofrequency in cosmetic dermatology. Dermatol Clin. 2014;32(1):79–90. https://doi.org/10.1016/j.det.2013.09.010. 54. Groen JW, Krastev TK, Hommes J, Wilschut JA, Ritt M, van der Hulst R. Autologous fat transfer for facial rejuvenation: a systematic review on technique, efficacy, and satisfaction. Plast Reconstr Surg Glob Open. 2017;5(12):e1606. https://doi.org/10.1097/ gox.0000000000001606. 55. Marten TJ, Elyassnia D.  Fat grafting in facial rejuvenation. Clin Plast Surg. 2015;42(2):219–52. https:// doi.org/10.1016/j.cps.2014.12.003. 56. Tzikas TL.  Lipografting: autologous fat grafting for total facial rejuvenation. Facial Plast Surg. 2004;20(2):135–43. https://doi. org/10.1055/s-­2004-­861754. 57. Atiyeh BS, Chahine F, Ghanem OA.  Percutaneous thread lift facial rejuvenation: literature review and evidence-based analysis. Aesthet Plast Surg. 2021;45(4):1540–50. https://doi.org/10.1007/ s00266-­020-­02095-­1. 58. Cobo R. Use of Polydioxanone threads as an alternative in nonsurgical procedures in facial rejuvenation. Facial Plast Surg. 2020;36(4):447–52. https://doi. org/10.1055/s-­0040-­1714266. 59. De Masi EC, De Masi FD, De Masi RD. Suspension threads. Facial Plast Surg. 2016;32(6):662–3. https:// doi.org/10.1055/s-­0036-­1597541. 60. Suh DH, Jang HW, Lee SJ, Lee WS, Ryu HJ. Outcomes of polydioxanone knotless thread lifting for facial rejuvenation. Dermatol Surg. 2015;41(6):720–5. https://doi.org/10.1097/dss.0000000000000368. 61. Tavares JP, Oliveira C, Torres RP, Bahmad F Jr. Facial thread lifting with suture suspension. Braz J Otorhinolaryngol. 2017;83(6):712–9. https://doi. org/10.1016/j.bjorl.2017.03.015. 62. Na HG, Jang YJ. Dorsal augmentation using alloplastic implants. Facial Plast Surg. 2017;33(2):189–94. https://doi.org/10.1055/s-­0036-­1598015. 63. Bartow MJ, Raggio BS.  Liposuction. In StatPearls. StatPearls Publishing Copyright © 2021, StatPearls Publishing LLC; 2021. 64. Regan JP, Casaubon JT.  Abdominoplasty. In StatPearls. StatPearls Publishing Copyright © 2021, StatPearls Publishing LLC; 2021. 65. Lobos N, Wortsman X, Valenzuela F, Alonso F. Color Doppler ultrasound assessment of activity in keloids. Dermatol Surg. 2017;43(6):817–25. https://doi. org/10.1097/dss.0000000000001052. 66. Kwitko GM, Patel BC. Blepharoplasty ptosis surgery. In StatPearls. StatPearls Publishing Copyright © 2021, StatPearls Publishing LLC; 2021. 67. Raggio BS, Asaria J. Open rhinoplasty. In StatPearls. StatPearls Publishing Copyright © 2021, StatPearls Publishing LLC; 2021.

Cutaneous Ultrasonography in Pediatric Dermatology

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Ana Isabel Rodríguez Bandera

Introduction Generalities The use of cutaneous ultrasonography is particularly appealing in children as it is a noninvasive and harmless diagnostic tool that does not require the child to remain isolated and still for an extended period of time. As pediatric dermatology covers a wide spectrum of diseases, this chapter focuses on a selection of disorders that are exclusive, more prevalent, or characteristic in childhood. In order to better understand the sonographic presentation of each entity, a clinico-histopathologic-­sonographic correlation will be offered. It is important to remember that, although sonographic features may be highly suggestive or characteristic of some disorders, the diagnosis can only rarely be made based solely on the imaging findings. However, one of the most important advantages that cutaneous ultrasonography offers is the possibility of a realtime and concomitant clinical and sonographic assessment. Sonographic descriptions discussed in this chapter are mostly based on images obtained with linear probes with variable frequencies up to 18–22 MHz. A. I. Rodríguez Bandera (*) Department of Dermatology, University Hospital La Paz, Madrid, Spain

Environment, Distraction, and Sedation Patient immobility is particularly important during cutaneous ultrasonography as the probe does not rest on the skin surface but remains suspended within the layer of gel above the lesion. This is particularly difficult to obtain when assessing young children unable to understand and follow instructions. Providing an optimized physical environment may be helpful. Newborns and infants might be more amenable to examination in a dark and quiet area, whereas toddlers and preschoolers may feel more at ease in a bright and colorful location. Natural sleep can be easily induced in newborns and infants by feeding them 20  min prior to the examination. Feeding during the examination or administering an oral sucrose solution can also be calming. Psychological preparation can reduce anxiety in patients old enough to comprehend the explanation. For example, demonstrating ultrasonography first on a patient’s doll or a parent can alleviate the fear of the unknown [1]. Audiovisual methods, including music, cartoon books, digital tablets, and video goggles, are also useful distraction techniques [1–4]. However, despite best efforts, constant crying or fighting movements can prevent quality images from being obtained [5, 6]. A retrospective study based on 543 exams showed that this is particu-

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larly problematic when assessing children aged between 4 and 12 months and lesions located in periorificial areas, anterior neck fold, or scalp, especially when a Doppler exam is required [1]. Thus, occasionally, depending on the type, ­location and extent of the dermatologic condition and the age o the patient, sedation may be helpful, especially if the test cannot be delayed to an age in which the patient will be more collaborative. Chloral hydrate, 50  mg/kg, administered orally 30 min before examination, has been proposed as a safe option. It does not require the presence of an anesthetist, but children need monitoring, which can be done by using the modified Aldrete score [7, 8].

Nonvascular Tumors and Hamartomas Nonvascular Tumors We present a selection of benign and malignant tumors, which present in the dermis and/or hypodermis and are exclusive or more commonly seen in pediatric patients or are a clue for the diagnosis of genetic syndromes.

Benign Tumors Neurofibroma Neurofibroma is the most prevalent benign peripheral nerve sheath tumor and represents a diagnostic criterion for neurofibromatosis type 1. There is a wide spectrum of clinical presentations, and several classifications have been published. They are usually divided according to the depth (cutaneous/subcutaneous) and growth pattern (localized/diffuse/plexiform). Small to large sizes and flat, sessile, globular, and pedunculated appearances have been described [9]. They can be skin colored and hyper- or hypopigmented and often demonstrate a soft consistency. Plexiform neurofibromas usually exhibit complex shapes, follow nerve trajectories, and can be particularly large in size. They can cause pain, neurological dysfunction, and disfigurement [10] and can undergo malignant transformation, with patients

A. I. Rodríguez Bandera

presenting with multiple plexiform neurofibromas at a higher risk [11]. Histopathologically, it is composed of cells of diverse lineages, including Schwann cells, mast cells, endoneurial fibroblasts, and perineurial cells, embedded in an abundant collagen-rich extracellular matrix [12]. Cells from various skin adnexa (i.e., hair follicles, eccrine sweat glands, sebaceous glands) and adipocytes can also be seen [9]. Sonographic images also reflect the clinical and histological heterogeneity of neurofibromas, with different lesions showing different morphologies, echogenicities, and locations (Fig.  23.1). Nodular or localized neurofibromas are the most frequently described, presenting as well-defined, ovoid or fusiform, mostly hypoechoic lesions, located in the dermis, hypodermis, or subfascial layers. The target sign is characteristic and reflects the tendency for these tumors to accumulate collagen fibers in the center (hyperechoic) and myxomatoid tissue in the periphery (hypoechoic) [13, 14] (Fig. 23.1c). Diffuse neurofibromas present as ill-defined, hypoechoic or heterogeneous, dermal or hypodermal lesions [15]. Plexiform neurofibromas have been described as a set of multiple hypoechoic nodules separated by hyperechoic tissue, with a serpentine-­ like arrangement, following the course of a nerve and its branches (Fig. 23.1e), in the dermis or deeper [11, 13, 16–18], often giving a “bag of worms” appearance [14]. Neurofibromas commonly share some sonographic findings. The presence of triangular or linear lateral extensions, corresponding to the underlying nerve, is characteristic [17] (Fig. 23.1d). While this feature is highly suggestive of a neurofibroma, it can also be found in other neural tumors [19]. Posterior enhancement is also frequently seen [17], as expected for a highly cellular tumor [20] (Fig.  23.1a). Color Doppler mode usually shows a hypovascular tumor, with no [21] or mild vascularity, mostly peripheral [18]. High-frequency ultrasonography (HFUS) can identify neurofibromas in patients with neurofibromatosis type 1 and be used for serial monitoring [21]. Identification of calcifications, anechoic areas suggestive of hemorrhage or necrosis,

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Fig. 23.1  Neurofibromas, high-frequency ultrasonography (HFUS), using 18 MHz. (a) Pedunculated cutaneous nodular homogeneously hypoechoic NF. Notice the posterior reinforcement and lateral acoustic shadows. (b) Sessile-flat cutaneous localized heterogeneously hypoechoic NF. Notice the characteristic lateral prolongation (star). (c) Scrotal hypodermal nodular NF. Notice the characteristic target sign: hyperechoic center and

hypoechoic periphery. (d) Hypodermal nodular NF. Notice the lateral spike or prolongation (star). Reuse permitted by Editorial Médica Panamericana [179]. (e) Hypodermal plexiform NF following the course of the superficial peroneal nerve. Reuse of Fig. 23.1d permitted by Editorial Médica Panamericana [179]. Reuse of Fig. 23.1e permitted by Wiley [22]

peripheral edema, or infiltration of adjacent tissues should raise suspicion of malignant peripheral nerve tumor [22, 23].

Sonographically, it usually appears as a hypodermal, well-defined, homogeneously hyperechoic mass, representing both the fat component and the fibrous septa. Different amounts and sizes of hypoechoic areas, representing the myxoid stroma, can be seen [14, 18, 24, 25]. Interestingly, unlike higher fat content lesions, such as simple superficial lipomas, which tend to be hypo- or isoechoic, the admixture of fat with other soft-­ tissue interfaces results in an increased echogenicity [26]. In color Doppler mode, mild internal vascularity is often visible [18]. Lipoblastomatosis refers to a more infiltrative presentation that can involve the underlying musculature [18] and manifest as a poorly circumscribed lesion [24].

Lipoblastoma Lipoblastoma is a rare benign tumor of embryonal fat, representing 30% of the fat-containing tumors in children. It typically appears within the first 3 years of life [14, 24, 25], and rarely beyond 8 years of age. The age of presentation helps differentiating lipoblastoma from liposarcoma, with the latter being extremely rare in children younger than 5 years [18, 26]. Clinically, it manifests as a slow-growing mass [25], but more rapid than a lipoma [18]. The limbs are the most frequent location [14, 25]. Histopathologically, it is a lobulated tumor composed of lipoblasts, mesenchymal cells, and mature adipocytes organized in lobules separated by fibrous septa and irrigated by a plexiform capillary network. Variable amounts of myxoid stroma can be found within the tumor [25].

Pilomatricoma Also called pilomatrixoma, this is a benign tumor usually occurring during the first two decades of life. It originates from follicular matrical cells and has been associated with mutations in CTNNB1 that encodes β-catenin, which promote apoptosis

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[27]. The development of multiple pilomatricomas is rare and should prompt the suspicion of a genetic syndrome (i.e., familial adenomatous polyposis syndromes, myotonic dystrophy, Turner syndrome), particularly if there are more than six lesions [28]. It presents as an asymptomatic, slowly growing, skin-colored or bluish nodule, most commonly located on the head, neck, or upper limbs (Fig. 23.2a and  23.4a). Occasionally, the dermis overlying a pilomatricoma develops anetoderma and the lesion can acquire a bullous appearance (Fig. 23.5a). On palpation, it is characteristically firm, often described as “rock hard,” multifaceted, and mobile. Histopathologically, in early stages, intermediate-­ size, nucleated basaloid germinative matrical cells, arranged in the periphery, progressively transition into enucleated dead cells, known as shadow cells, that accumulate in the center. They produce keratinized debris and, with time, undergo dystrophic calcification or ossification (Fig.  23.2b). In addition, a foreign-body granulomatous response may be present. At a later stage, the basaloid component may be minimal or absent [29].

a

Ultrasonographic images also show pilomatricoma dynamism, and efforts have been made to classify them into patterns [30] and to correlate findings with the duration of symptoms [31]. It usually presents as a well-defined and oval-­ shaped lesion, although ill-defined tumors and round or irregular morphologies have been described [32]. It is predominantly located in the hypodermis, but must be in contact with the dermis, where it originates [33] (Figs. 23.2c, 23.3a, and 23.4b). Its echogenicity is often heterogeneous [32]. The presence of a hypoechoic rim, internal echogenic foci, and a posterior acoustic shadow is highly suggestive of pilomatricoma [32, 34] and confers the characteristic targetoid sonographic appearance [35] (Fig.  23.3). The absence of any or all of these three features makes the diagnosis less likely, but not impossible [32]. The hypoechoic rim is thought to represent the connective tissue capsule [32], and peripheral basaloid cells may also contribute. The hyperechoic foci correspond to calcifications or areas of ossification and are responsible for the posterior acoustic shadow (Fig.  23.2d, 23.3 and 23.4b). They can vary in quantity and

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Fig. 23.2  Pilomatricoma, large lesion. (a) Clinical picture: firm large nodule on the cheek of an 8-year-old girl. (b) Histopathology: hematoxylin-eosin (x2): Well-­ defined, irregularly ovoid tumor containing enucleated cells, keratinized debris (star), and foci of calcification (arrow). Courtesy of Maria Beato Merino, Department of Pathology, University Hospital La Paz. (c) HFUS (18 MHz), transverse plane: Well-defined, echogenically

heterogeneous tumor, mostly located in the hypodermis but abutting the dermis. Notice the hypoechoic halo and the posterior acoustic shadow. (d) HFUS (18 MHz), longitudinal plane: well-defined irregularly ovoid tumor abutting the dermis. Notice the echogenic areas likely corresponding to keratinized debris (star) and the highly hyperechoic foci corresponding to calcification (arrow)

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Fig. 23.2 (continued)

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Fig. 23.3 Pilomatricoma, targetoid appearance. (a) HFUS (19 MHz) shows a well-defined rounded hypodermal lesion connected to the deep dermis. Notice the peripheral hyperechoic halo, the echogenic center, and the posterior acoustic shadow. This is the most classic sono-

graphic presentation of pilomatricoma, with a characteristic targetoid appearance. (b) HFUS (22  MHz), color Doppler mode, of the same lesion. Notice the most hyperechoic structures corresponding to calcifications (arrowhead)

distribution, from scarcely scattered dots (Fig.  23.2d) to large arcuate structures occupying most of the lesion [34] (Fig.  23.4b). The presence of alternating highly hyperechoic dots (corresponding to calcifications or areas of ossification) and mildly echogenic dots (likely representing areas of debris or keratinization resulting from the evolution of ghost cells) is highly sug-

gestive (Figs.  23.2d and 23.3b). Peritumoral hyperechogenicity, thought to represent chronic foreign-body inflammatory reaction [31], has been described as a rather common but nonspecific finding [32] (Fig. 23.2c). Doppler color mode shows internal blood flow in 50–80% [31, 32, 34] of lesions, most commonly located at the periphery. This is in

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Fig. 23.4 Pilomatricoma, arciform calcification. (a) Clinical picture of pilomatricoma on the forehead of a teenager. Palpable subcutaneous hard nodule. (b) HFUS (18 MHz) shows an arciform highly hyperechoic structure

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abutting the deep dermis and generating a posterior acoustic shadow, corresponding to a large calcification. This is the second most common pattern of pilomatricoma

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Fig. 23.5  Bullous pilomatricoma. (a) Clinical picture shows a tumor located on the upper arm with a bulla-like or anetodermic surface. (b) HFUS (22  MHz), B-mode, demonstrates a characteristic image of a pilomatricoma: a well-defined, dermo-hypodermal tumor with a hypoechoic

rim (arrowhead) and a heterogeneous hyperechoic center producing a posterior acoustic shadow (star). Notice the conspicuous edema of the dermis over the tumor (arrow). (c) Color Doppler mode demonstrates abundant vascularity within the tumor. Reuse permitted by Wiley [33]

keeping with the expected presence of stroma, containing blood vessels, forming a rim in the margins and scattered islands in the center of the lesion. These findings facilitate the differential diagnosis with follicular cysts, which are avascular by definition. Vascularity within pilomatricomas can be strikingly abundant [36] (Fig. 23.5c). In the author’s experience, the presence or absence of vascularity can be used to guide patients’ expectations and treatment decisions, with highly vascularized tumors more likely to continue growing. Anetodermic or bullous pilomatricoma (also called cystic pilomatricoma) appears to abut a distorted dermis, which can appear thickened,

with hypoechoic or anechoic lagoons, most likely representing edema and ultimately responsible for the bullous appearance [33] (Fig. 23.5). Syringocystadenoma Papilliferum Syringocystadenoma papilliferum is a rare benign sweat gland tumor, which can arise within a nevus sebaceous [37]. Most are congenital or develop before puberty [38]. It mostly involves the head or neck, with the scalp being the most frequent location, but it has also been described in other areas [39]. It is usually asymptomatic and presents as hairless, skin-colored to erythematous, solitary or grouped papules, plaques, or nodules, sometimes with a linear arrangement,

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Fig. 23.6  Syringocystadenoma papilliferum. (a) Clinical picture. Congenital erythematous, linear, and alopecic lesion, with a nodular eroded component (arrow), in the occipital area of the scalp of a 2-month-old baby. (b)

HFUS (18  MHz), B-mode, of the nodular component (arrow). Well-defined, dermo-hypodermal, mostly hypoechoic lesion with irregular borders. (c) Color Doppler mode shows significant intralesional vascularity

and often verrucous, crusted, or eroded [40] (Fig. 23.6a). It tends to grow slowly. Histopathologically, it is composed of papillary projections and ducts of various sizes and shapes resulting from invaginations in the surface epithelium, with which it remains connected. The stroma of the papillary processes contains connective tissue, dilated vessels, and inflammatory cells, mainly plasma cells [41]. Sonographically, it presents as a well-defined, mostly hypoechoic lesion with irregular borders (Fig. 23.6b). Color Doppler mode can show significant vascularity [42] (Fig. 23.6c).

Sonographically, it usually appears as an ill-­ defined or, less likely, lobulated tumor, located in the dermal and hypodermal layer or only in the hypodermis [45]. In concordance with its heterogeneous histologic appearance, it is heterogeneously hyperechoic, often containing trabeculated hypoechoic bands and a peripheral hypoechoic rim [18, 45]. Regarding its echostructure, it can show a “layered” appearance or a “serpiginous” pattern [14, 45]. Color Doppler mode usually shows low internal vascularity [14, 18, 45].

Fibrous Hamartoma of Infancy (FHI) Despite its name, fibrous hamartoma of infancy has been classified as a fibroblastic/myofibroblastic tumor by the WHO [43]. It typically occurs in children younger than 2  years, and around 20% of cases are congenital. The trunk, axillae, extremities, and genital regions are the most frequently affected [18, 44]. It generally presents as a solitary, poorly circumscribed, skin-­colored nodule, with no epidermal changes [18]. Multiple lesions, hyperpigmentation, hypertrichosis, and hyperhidrosis have been reported [44]. Histopathologically, it is described as a poorly circumscribed mass with a characteristic triphasic appearance due to the presence of mature fibroblastic-myofibroblastic cells and variable amount of collagen bundles, mature adipose tissue, and an immature mesenchymal component [18, 44].

Infantile Myofibroma Myofibroma is classified as a pericytic (perivascular) tumor [43]. It occurs more frequently in neonates or infants younger than 2 years of age [46]. Three main presentations have been recognized: the solitary form, the most frequent, involving the skin and, sometimes, the subcutaneous tissue; the multicentric variant, affecting the skin, subcutis, muscle, and bone; and the generalized form, which is associated with visceral lesions [46]. Cutaneous tumors usually present as firm, red-to-brown nodules or plaques, sometimes with central necrosis or ulceration [46]. The solitary and multicentric variants have a good prognosis, with spontaneous regression often occurring during the first few years after the diagnosis. The presence of visceral lesions is associated with a poorer prognosis and may be fatal [46]. Histopathologically, infantile myofibroma displays a nodular and well-circumscribed appearance. Microscopically, it is characterized

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by the presence of two distinct myofibroblastic cell aggregates: spindle cells arranged in fascicles within a collagenous stroma, and more ­primitive and smaller round blue cells surrounding thin-walled and staghorn-like vessels with a hemangiopericytoma-like pattern [47]. Areas of cystic degeneration, necrosis, fibrosis, and massive hyalinization can be found [46]. Ultrasonography of cutaneous, subcutaneous [47, 48], and intramuscular [14] lesions has shown a well-demarcated, irregularly ovoid [14, 48] or multilobular [47] lesion. It is mostly hypoechoic, due to its high cellular component, but it usually exhibits some hyperechoic areas [14, 47, 48], likely representing areas of interlacing collagen fibers or fibrosis. It can produce a subtle posterior reinforcement [48]. Cystic degeneration or necrosis would be expected to appear as anechoic areas and calcifications as highly hyperechoic structures generating a posterior acoustic shadow. It has been described as a hypovascular tumor, with color Doppler mode showing none or few blood vessels, mostly at the periphery [14, 48]. Thus, HFUS facilitates the differential diagnosis of myofibroma with infan-

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Fig. 23.7  Superficial angiomyxoma. (a) Clinical picture: Pink papulo-nodule on the right lower back of a 14-year-­ old girl with Carney complex. (b) HFUS (18 MHz) shows a mostly hypoechoic lesion composed of three lobules, with a bell-like appearance, located in the dermis and hypodermis. Notice the connection with the epidermis representing the clinically apparent “punctum.” (c) Histologically, hematoxylin and eosin stain (x2), a well-­ circumscribed, nonencapsulated tumor with a “bell-like” morphology, composed of myxoid matrix, mild-to-­

tile and congenital hemangiomas, and hypervascular soft-tissue malignant tumors, which are the main differential diagnoses. Superficial Angiomyxoma Superficial angiomyxoma can prompt the diagnosis of Carney complex, an autosomal dominant multiple neoplasia and lentiginosis syndrome. In addition to superficial angiomyxomas, patients with Carney complex can develop other tumors, such as cardiac myxomas, and thyroid, ovarian, pancreatic, and liver cancers, as well as endocrinopathies, such as acromegaly and adrenocortical disease, hence the importance of an early diagnosis [49]. The clinical appearance of superficial angiomyxoma is not distinctive. It has been described as a skin colored or pinkish [50] polypoid lesion, papulo-nodule or nodule, more frequently located on the trunk and head and neck region [51] (Fig. 23.7a). Histopathologically, it has been described as a well [50] or poorly [51] circumscribed, dermo-­ hypodermal, lobulated or multilobulated [52] tumor composed of a prominent myxoid (muci-

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moderate cellularity, including several basaloid entrapped nests, prominent capillaries, and a few collagen bundles. Notice that the collagenous stroma is more abundant in the most superficial part or tip of the lesion, which corresponds to the most hyperechoic area on the sonographic picture. Reuse of Fig. 23.7a and b permitted by Editorial Medica Panamericana [179]. Figure 23.7c is courtesy of Maria Beato Merino, Department of Pathology, University Hospital La Paz

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Fig. 23.8  Juvenile xanthogranuloma. (a) Clinical picture shows a yellowish nodule on the scalp of a 2-year-old boy. (b) HFUS (18 MHz) demonstrates a well-defined hypoechoic dermo-hypodermal tumor with irregular borders

nous) matrix containing moderate to scarce cellularity, mostly fibroblasts, blood vessels, and few collagen bundles [51] (Fig. 23.7c). Sonographically, although literature is limited, in the author’s experience and consistent with the described histopathological findings, superficial angiomyxoma appears as a dermo-­ hypodermal, well-defined, lobulated, mostly hypoechoic tumor, which produces an acoustic posterior reinforcement. The majority of elements that compose the tumor (myxoid and homogeneous cellularity) transmit ultrasound well, which explains the overall low echogenicity. The posterior reinforcement is generated by the change of impedance from the tumor to the hypodermis, as the latter reflects more of the ultrasound. Interestingly, the deeper lobules of the tumor seem to be larger, conferring a bell-like or pear-like appearance. Thus, the clinically visible tumor may only represent the “tip of the iceberg,” which highlights the benefit of HFUS in the pre-­surgical delimitation (Fig. 23.7). In color Doppler mode, blood vessels may be visible within the lesion, which is not seen in cysts.

face, most frequently located on the head and neck. Color varies from pink-red in early stages to yellow-orange-brown in older lesions (Fig.  23.8a). On dermoscopy, the “setting-sun pattern,” consisting of central orange areas with pale-yellow areas and an erythematous halo, is characteristic. Peripheral linear telangiectasias have been described. The presence of multiple lesions should prompt investigation of systemic involvement [53]. Histopathologically, it presents as a nonencapsulated, well-circumscribed lesion occupying the dermis and the uppermost part of the subcutis. It is composed of a dense sheetlike histiocytic infiltrate, with characteristic Toutontype multinucleated giant cells visible in around 85% of lesions [53]. Sonographically, it has been described as a well-defined, ovoid, and homogeneously hypoechoic dermal nodule [54, 55] (Fig. 23.8b). It displaces the epidermis upward [55] and can also extend into the upper hypodermis. Color Doppler mode can detect occasional thin blood vessels within the tumor, with low-velocity arterial flows [22, 55].

Juvenile Xanthogranuloma Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis and typically occurs in young children. It usually presents as a solitary, asymptomatic, well-­ demarcated papule or nodule with a smooth sur-

Malignant Tumors Metastatic Neuroblastoma Neuroblastoma represents one of the most common solid malignancies during infancy [56, 57]. It arises from the neural crest cells forming the

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Fig. 23.9  Metastatic neuroblastoma. (a) Clinical picture shows a skin-colored subpalpebral swelling (arrow). (b) HFUS (19 MHz) reveals a well-defined hypoechoic lesion creating a posterior acoustic enhancement. Notice intral-

esional hyperechoic dots that may represent microcalcifications (arrow). Color Doppler mode showed sparse blood vessels within the tumor. Reuse permitted by Wiley [22]

sympathetic nervous system, most often in the adrenal medulla [22]. Metastasis is common, particularly to the skin, where they present as asymptomatic subcutaneous nodules with a blue hue [58]. Thus, metastatic neuroblastoma is one of the differential diagnoses of “blueberry muffin baby.” The periorbital region is often involved, usually bilaterally, potentially resulting in proptosis, periorbital bruising (“raccoon eyes”), periorbital swelling, and/or strabismus [22, 58]. Both periorbital and corporal lesions have been mistaken with infantile hemangioma [59, 60]. The prognosis is highly variable, even with metastatic disease, ranging from spontaneous regression to a fatal outcome [59]. Histopathologically, cutaneous neuroblastoma usually presents as a relatively well-­ circumscribed tumor. As it arises from pluripotent neural crest cells, with a broad spectrum of differentiation, individual tumors are composed of a mixture of different kinds of neural and Schwannian cells with different degrees of maturation, and the ratio of cells to stroma will decrease with maturation. The most commonly described histological picture is that of a highly cellular and stroma-poor tumor, mainly composed of small blue cells, arranged in rosettes with a fibrillary material in the center

[61]. Microcalcifications are frequently seen, especially within abdominal tumors, and areas of necrosis have been described [62]. Ultrasound descriptions available in the literature, mostly based on primary adrenal tumors, also reflect the histologic heterogeneity of these tumors [63, 64]. Cutaneous tumors with high cellularity are expected to be mostly hypoechoic, while images with more echogenic areas may represent tumors richer in stroma. Punctate microcalcifications can be seen [22] (Fig.  23.9b). It is usually described as a hypovascular tumor, with scattered thin intralesional blood vessels [22, 59] or only peripheral vascularity [14]. In such cases, color Doppler mode has helped differentiating metastatic neuroblastoma from proliferating infantile hemangioma, which are hypervascular [22, 59]. However, neuroblastoma exhibiting a more profuse vascularity has been reported [42], and in those cases sonographic differential diagnosis can be extremely challenging. Plexiform Fibrohistiocytic Tumor Plexiform fibrohistiocytic tumor is a rare soft-­ tissue tumor of intermediate malignancy (rarely metastasizing), classified as a so-called fibrohistiocytic tumor [43]. It mostly affects children and

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Fig. 23.10  Congenital plexiform fibrohistiocytic tumor. (a) Clinical picture shows an ill-defined ulcerated and indurated erythematous plaque on the dorsum of a newborn. (b) HFUS (18  MHz) demonstrates an ill-defined ulcerated infiltrative tumor with a heterogeneous echogenicity. The low echogenicity in the upper part of the

young adults, with few congenital cases described [65]. Clinically, it usually appears as a painless and slow-growing, skin-colored, erythematous or violaceous nodule or firm plaque, although it can present as a painful and ulcerated lesion [65] (Fig. 23.10a). The upper extremities are the most common location, but it may also involve the lower extremities, trunk, and head and neck region. Histopathologically, it appears as a poorly circumscribed, dermo-hypodermal tumor composed of small cellular nodules or fascicles arranged in a plexiform pattern within variable fibrous stroma. The nodules are composed of histiocyte-­ like cells with multinucleated osteoclast-like giant cells in the center and fibroblast-like cells in the periphery [65, 66]. Ultrasound shows a well- [67] or ill-defined [65] lesion involving the dermis and/or hypodermis, with or without extension into the skeletal muscle. It can present as a mostly hypoechoic [42, 67] or echogenically heterogeneous [65] tumor. Color Doppler mode shows moderate [65] to marked internal vascularity [42] (Fig. 23.10).

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lesion may be related to necrosis clinically manifesting as ulceration. Notice how the tumor appears to extend between and beyond the metatarsals (arrowhead). (c) Color Doppler mode demonstrates blood vessels within the tumor. Further details of this case are available in the literature [65]

tissue malignancies in infancy [14] and about 1.6% of all malignant solid tumors in this age group. The extremities, trunk, head and neck region, and, more rarely, retroperitoneum can be involved [14, 69]. It manifests as a rapidly growing, painless mass, which can present with a tense, erythematous, or ulcerated surface, mimicking a vascular lesion [69]. Histopathologically, it is identical to classic adult fibrosarcoma, but is associated with a much more favorable prognosis. Macroscopically, it is poorly circumscribed and lobulated. Microscopically, it is a highly cellular tumor, composed of monomorphic primitive hyperchromatic ovoid-to-spindle cells arranged in tight fascicles within a variably collagenous stroma. It is diffusely infiltrative and may display prominent dilated hemangiopericytomalike vasculature, focal necrosis, and hemorrhage [68, 69]. Sonographically, it appears as a poorly circumscribed, infiltrative mass involving the subcutaneous tissue [14] and/or the muscular layer [69]. Echogenically, it has been described Infantile Fibrosarcoma as a heterogeneous, but mostly hypoechoic, Infantile fibrosarcoma is a rare fibroblastic malig- tumor [14, 69]. Vascularity can be variable nant neoplasm, usually found in children younger [14], but it is more commonly hypervascular than 2 years, and over a third of cases are present and may be difficult to differentiate from an from birth [14, 68]. It accounts for 12% of soft-­ infantile hemangioma [69].

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B-Cell Lymphoblastic Lymphoma (BcLL) Lymphoblastic lymphoma is the second most common type of non-Hodgkin lymphoma in children [70]. BcLL is primarily a disease of childhood, with 75% of cases occurring in patients younger than 6  years of age [70]. Osteolytic bone lesions (26%) and cutaneous or subcutaneous lesions (23%) are the most common sites of disease [70]. In the skin, it typically presents as a nodule on the head and neck, often clinically mistaken for a lipoma, dermoid cyst, or insect bite [22] (Fig. 23.11a). Asymptomatic and firm ipsilateral cervical lymphadenopathies may be present concomitantly [71]. Histopathologically, it is composed of a dense, monomorphic dermal and subcutaneous infiltrate

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Fig. 23.11  B-cell lymphoblastic lymphoma, cutaneous lesion. (a) Clinical picture shows an erythematous mass on the scalp of an 8-year-old boy. (b) HFUS (18  MHz) demonstrates an ill-defined, heterogeneous but mostly

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of small- to medium-sized lymphocytes, under a Grenz zone [71] (Fig. 23.11c). Sonographically, as expected for a tumor with a high and monomorphous cellularity [20], it has been described as a predominantly hypoechoic lesion, occupying both the dermis and hypodermis, with ill-defined margins and no increased flow in Doppler mode [71] (Fig.  23.11b). The Grenz zone will appear as an echogenically preserved upper dermis. When assessing lymph nodes, sonographic signs of malignancy include blurred margins, round shape, loss of central echogenicity, structural changes (cortical nodules or intranodal necrosis, reticulation, and matting), and a peripheral, mixed (central and peripheral), or chaotic vascularity [71] (Fig. 23.12).

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Fig. 23.12 B-cell lymphoblastic lymphoma, cervical lymphadenopathies. (a) Clinical picture of asymptomatic, skin-colored, firm, cervical lymphadenopathies. (b) HFUS (18 MHz) shows a round, hypoechoic lymphadenopathy with no recognizable central hyperechoic medulla

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hypoechoic, dermal, and hypodermal lesion. (c) Hematoxylin and eosin (H&E) stain (x2) shows a dense, monomorphic, dermal, and subcutaneous infiltrate under a Grenz zone. Reuse permitted by Wiley [71]

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and non-hilar vascularity. (c) HFUS (18  MHz) demonstrates matting or fusion of lymphadenopathies. Reuse permitted by Wiley and Editorial Medica Panamericana [71, 179]

23  Cutaneous Ultrasonography in Pediatric Dermatology

Hamartomas Hamartomas are benign proliferations composed of mature or immature cellular elements native to a site, in an aberrant proportion. Although they can present in a tumorlike fashion, they are not authentic neoplasms.

 evus Sebaceous of Jadassohn N Nevus sebaceous of Jadassohn is one of the most common hamartomas. It is a postzygotic mosaic due to an activating mutation in the HRAS or KRAS gene, leading to cell growth secondary to activation of the MAPK signal transduction pathway [72]. Clinically, it presents at birth as a thin, alopecic, yellow-orange, linear plaque, most frequently located on the scalp or face. During puberty, likely influenced by hormones, it can become thicker or nodular, due to an increase in the number of sebaceous glands (Fig.  23.13a). Large blaschkoid lesions may be a sign of nevus sebaceous syndrome (Schimmelpenning syndrome), which is associated with cerebral and skeletal anomalies. There is an increased risk of developing a neoplasm within the lesion, including benign (trichoblastomas, sebaceomas, and syringocystadenoma papilliferum) and malignant tumors (basal cell carcinoma, sebaceous carcinoma, apocrine carcinoma) [72].

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Fig. 23.13  Nevus sebaceous of Jadassohn. (a) Clinical picture shows a linear, mamillated thick alopecic plaque with a light orange color on the scalp of a teenager. (b)

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Histopathologically, it is characterized by the presence of immature hair follicles and an increase in sebaceous and ectopic apocrine glands in the dermis [72]. The clinical thickening of the lesion during puberty corresponds with an increase in the number of sebaceous glands, which occupy not only the deep dermis but also the superficial dermis [73]. The epidermis usually shows acanthosis and papillomatosis [72]. Sonographically, it is characterized by an increase in dermal thickness, conferring a band-­ like appearance [74], and a decrease in echogenicity. The higher the number of pilosebaceous units and apocrine glands, the thicker and more hypoechoic the lesion will appear (Fig.  23.13b). The epidermis can appear thicker and undulated [74]. Color Doppler mode may show increased internal vascularity, with slow-flow vessels [74]. As the lesion is exclusively located in the dermis, very-high-frequency devices will provide better resolution of the entire lesion. They allow the differentiation of pilosebaceous units and sebaceous and apocrine glands. Sebaceous glands appear as hyperechoic oval-shaped structures, and apocrine glands present as small oval structures, adjacent to the pilosebaceous units, with a “pseudo-ovary” appearance (mixed hypoechoic and anechoic zones) [74].

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HFUS (18 MHz) demonstrated a hypoechoic mamillated conspicuous thickening of the dermis

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 utaneous Smooth Muscle Hamartoma C (CSMH)/Becker’s Nevus CSMH and Becker’s nevus share clinicopathologic characteristics. It remains controversial if they represent distinct entities or, more likely, polar forms of a spectrum of dermal smooth muscle hyperplasia [75, 76]. Cutaneous smooth muscle hamartoma is usually congenital, while Becker’s nevus typically appears during adolescence and has been described as an androgen-­ dependent lesion [76]. Clinically, CSMH usually presents as a skin-colored or slightly hyperpigmented, hypertrichotic patch or plaque, which may be composed of follicular papules [75] (Fig. 23.14a). A pseudo-Darier sign is characteristic: stroking the lesion causes temporary elevation or piloerection due to contraction of arrector pili smooth muscles [75, 77]. The hyperpigmentation and hypertrichosis are typically more conspicuous in Becker’s nevus [75, 76]. Histologically, both are characterized by an excess of haphazardly oriented smooth muscle bundles in the mid-deep dermis [75–77]. Sonographically, in the author’s experience, there is a hypoechoic thickening of the mid- and deep dermis. Hypertrichosis can be easily identifiable (Fig.  23.14b). Color Doppler mode does not show increased blood flow.

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Fig. 23.14  Cutaneous smooth muscle hamartoma. (a) Clinical picture shows a slightly hyperpigmented and hypertrichotic patch on the left scapula of a 4-year-old

Cutaneous Fibrolipomatous Hamartoma Cutaneous fibrolipomatous hamartoma is considered a rare condition but is probably underreported. Most cases are sporadic, although familial cases, with a proposed autosomal dominant or X-linked pattern of inheritance, have been described. An occasional association with Gardner syndrome and tuberous sclerosis has been reported. Although its pathogenesis is unknown, an alteration in the involution of the physiologic subcutaneous hypertrophy of the fetal heel during the final months of pregnancy and first months of life is the most popular theory [78]. It has received many descriptive names, including precalcaneal congenital fibrolipomatous hamartoma, bilateral congenital adipose plantar nodules, bilateral congenital fatty heel pads, and pedal papules of the newborn. The most common clinical presentation is that of congenital, bilateral, and symmetric, skin-colored, soft nodules located on the inner aspects of the heels. However, unilateral and retrocalcaneal lesions have been described, and they can be noted after birth [78] (Fig. 23.15a). Histopathologically, it consists of well-defined lobules of mature fat cells separated by fibrous

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boy (arrow). (b) HFUS (18  MHz) demonstrated a hypoechoic thickening of the mid- and deep dermis (bracket). Notice the hypertrichosis

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Fig. 23.15  Cutaneous fibrolipomatous hamartoma. (a) Clinical picture of a congenital skin-colored soft mass on the retrocalcaneal region of a 2-year-old boy. (b) HFUS (18 MHz) demonstrates a thickening of the dermis (arrow) with alternating hypoechoic and hyperechoic bands in the

reticular dermis. (c) Hematoxylin and eosin (H&E) stain (x2) shows well-defined lobules of mature fat surrounded by fibrous septa in the mid- and deep dermis. Reuse permitted by Wiley [78]

septa observed in the mid- and deep dermis with no neural involvement [78] (Fig. 23.15c). Sonographically, it manifests as a non-­ compressible thickening of the reticular dermis, extending towards the hypodermis, composed of alternating hypoechoic and hyperechoic bands or hypoechoic islands separated by hyperechoic bands in a honeycomb pattern. The hypoechoic bands or islands represent the mature adipose tissue lobules, while the hyperechoic bands represent collagenous septa (Fig.  23.15b). In color Doppler mode, no increased intralesional blood flow is expected [78, 79].

previa, placental abruption, umbilical cord prolapse, obstetric trauma, macrosomia, fetal congenital heart disease, seizures, anemia, meconium aspiration, therapeutic hypothermia) [80, 81]. Clinically, it manifests as single or multiple painful red-brown or violaceous indurated plaques or nodules, commonly located on the back, buttocks, proximal extremities, and cheeks [80] (Fig. 23.16a). Fever is present in over half of the cases [82]. Potential complications include hypercalcemia, hypertriglyceridemia, hypoglycemia, and thrombocytopenia [80, 82]. Histopathologically, it manifests as a  lobular panniculitis with a mixed cell infiltrate composed of lymphocytes, histiocytes, multinucleated giant cells, and occasional eosinophils. The presence of birefringent needle-shaped clefts arranged in radial array within adipocytes, histiocytes, and multinucleated giant cells is characteristic [80]. Sonographically, it appears as a moderately well-defined [83, 84] to ill-defined [82], diffuse [82, 84–86] or lobular [81] hyperechogenicity of the hypodermis (Fig. 23.16b). Anechoic lacunar areas, likely representing areas of necrosis, may be seen [81]. Interfaces creating posterior acoustic shadows may be found and reflect the presence of calcifications [86]. Color Doppler mode shows a slight to moderately increased vascular flow [81, 82, 86] (Fig. 23.16c).

Inflammatory Lesions  ubcutaneous Fat Necrosis S of the Newborn (SCFN) SCFN is a rare and transient mosty lobular panniculitis, usually affecting term or post-term infants during the first weeks of life and resolving within weeks or months without sequelae [80, 81]. Although the exact etiology is unknown, it has been associated with maternal, fetal, or birth complications, especially those leading to fetal or neonatal hypoxia or asphyxia (maternal diabetes, maternal hypertension, preeclampsia, placenta

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Fig. 23.16  Subcutaneous fat necrosis of the newborn. (a). Clinical picture shows an erythematous mass on the right shoulder of a 1-week-old full-term newborn. (b) HFUS (18  MHz) demonstrates a lobular homogeneous

increase of the echogenicity in the hypodermis. (c) Color Doppler mode shows subtle increased vascularity within the lesion

I diopathic Facial Aseptic Granuloma (IFAG)

Sonographic findings will depend on the stage of the lesion. During the active or inflammatory phase, the lesion appears as dermal, well defined, oval, with the major axis parallel to the skin surface, and mostly hypoechoic [87, 89, 90, 93, 94]. It may present with an illdefined or lobulated deeper margin [90, 95]. A posterior reinforcement or increase in the underlying hypodermis is usually seen and has been explained as a sign of subcutaneous inflammation or as a posterior ­reinforcement due to the change of impedance from the densely cellular inflammatory lesion and blood vessels, which transmit ultrasound easily, to the subcutaneous tissue [90, 96] (Fig.  23.17b). Color Doppler mode shows increased blood flow, both intra- and perilesional, most prominent in and around the deepest part of the lesion [90, 91] (Fig.  23.17c). Upon resolution, the

IFAG is a self-resolving inflammatory lesion, primarily affecting children [87]. It is considered to belong to the spectrum of rosacea [88, 89]. Clinically, it presents as one or a few asymptomatic, red-to-violaceous, soft or elastic nodules, typically located on the cheek, within a triangle delimitated by the labial commissure, the lobule of the ear, and the inferior orbital margin [87, 90] (Fig. 23.17a). It is often misdiagnosed as an epidermoid cyst, pilomatricoma, odontogenic sinus, cutaneous leishmaniasis, or mycobacterial infection [22]. Histopathologically, it appears as a prominent granulomatous infiltrate in the dermis, mostly composed of aggregates of epithelioid histiocytes and occasional multinucleated cells, lymphocytes, and plasma cells [88, 91, 92].

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Fig. 23.17 Idiopathic facial aseptic granuloma. (a) Clinical picture shows an erythematous nodule on the cheek of a 6-year-old boy. (b) HFUS (18 MHz) demonstrates a dermal, well-defined oval-shaped lesion, with the major axis parallel to the skin surface. It is mostly

hypoechoic, with a subtle lobulated deep margin. Notice a mild increase in the echogenicity of the surrounding tissue or posterior acoustic reinforcement. (c) Color Doppler mode shows perilesional vascularity. Reuse permitted by Elsevier España [90]

thickness of the lesion decreases, the underlying hypodermis recovers its normal echogenicity, and the increased vascularity disappears [90]. Calcifications within the lesion have not been reported [87, 89–95].

foci of intralesional dystrophic calcification have been described [98] (Fig. 23.18e). Sonographically, it appears as an ill-defined, irregular, or stellate-shaped lesion, located in the hypodermis, commonly in contact with fascia [22, 98, 99]. It is mostly hypoechoic, as expected for a mucinous and high cell-to-matrix ratio lesion, and generates a posterior reinforcement [98]. The surrounding subcutaneous tissue may show a lobular or diffuse increase in echogenicity, likely representing the perilesional lymphocytic infiltrate [22]. Occasionally, shiny hyperechoic dots, representing calcium, can be found within the lesion [98]. Color Doppler mode usually does not show increased blood flow [98, 99] (Fig. 23.18b).

Subcutaneous Granuloma Annulare Subcutaneous granuloma annulare is a rare clinicopathologic variant of granuloma annulare, which appears more frequently in children. Clinically, it presents as an asymptomatic, skin-­colored to subtle brown, subcutaneous nodule, typically located on the anterior aspect of the lower leg, hands, head, or buttock [97] (Fig. 23.18a). Histopathologically, it consists of a nodular inflammatory infiltrate, located in the subcutis (Fig. 23.18c), composed of palisading histiocytes around central areas of collagen degeneration or necrobiosis and mucin [97] (Fig. 23.18d). Small

Linear Morphea Linear morphea is the predominant type of localized scleroderma in pediatric patients and

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Fig. 23.18 Subcutaneous granuloma annulare. (a) Clinical picture shows a subcutaneous skin-colored nodule on the pretibial aspect of the left leg (arrow). (b) HFUS (18 MHz) demonstrates an ill-defined hypoechoic avascular lesion in the hypodermis, in contact with the fascia. Notice the highly hyperechoic dots within the lesion, corresponding with the foci of calcification (see

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E). (c) Histopathology, hematoxylin and eosin (H&E) x2 shows a nodular inflammatory infiltrate in subcutaneous fat. (d) H&E x10 demonstrates palisading histiocytes around the central areas of collagen degeneration and mucin. (e) H&Ex20 shows basophilic deposits corresponding to calcium. Reuse permitted by Wiley [98]

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Fig. 23.19  Linear morphea. (a) Clinical picture shows sclerotic hyperpigmented patches on the legs of a 14-year-­ old girl. Veins appear more evident due to subcutaneous

atrophy. (b) HFUS (18 MHz) reveals significant atrophy of hypodermis, when compared with contralateral homologous area

is more commonly seen in children than adults [100]. It is characterized by a linear induration of the skin, following the lines of Blaschko, frequently associated with atrophy of the underlying subcutaneous tissue [101], hyperpigmentation, and/or hair loss (Fig.  23.19a). When affecting the extremities, it can be associated with arthralgias, joint contractures, and limb length discrepancy, and when involving the scalp or face (morphea “en coup de sabre” and Parry-Romberg syndrome), it can be associated with neurologic, ophthalmologic, and maxillofacial manifestations [100–102]. Histopathologically, morphea is an inflammatory sclerotic process characterized by an

increased number of collagen bundles, which are thick and parallel to the surface and appear firstly in the reticular dermis, with a surrounding inflammatory infiltrate. The epidermis can be ­ preserved or atrophic. Mucin may be present in early lesions. As it progresses, the sclerosis extends towards the papillary dermis and/or subcutis and, consequently, there is a progressive atrophy of adnexal structures and adipocytes. The blood supply diminishes as the lumen of the blood vessels narrows and their walls thicken. The number of inflammatory cells decreases [103, 104]. A study that included 64 biopsies of linear morphea showed moderate sclerosis (51.6%), which more commonly involved the

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reticular dermis and subcutis (51.6%) in comparison to other types of morphea, sparing the papillary dermis. The inflammatory infiltrate, mostly composed of lymphocytes and plasma cells, was usually in a perivascular and periadnexal location (90.6% and 85.9%, respectively) [105]. However, none of these findings appeared specific to this type of morphea [105]. Sonographically, when comparing with the homologous contralateral healthy skin, established lesions of linear morphea may appear with either a thickening or a thinning of the dermis [106], which will show an increased echogenicity where the sclerotic process has taken place (deep dermis ± papillary dermis), and with a striking thinning of the hypodermis, which can almost disappear [22, 107] (Fig.  23.19b). As a sign of subcutaneous sclerosis, the echogenicity of the remaining hypodermis can be increased and become similar to the echogenicity of the dermis, erasing the dermo-hypodermal junction [107, 108]. The dermis usually shows a decreased echogenicity. Elastography ultrasound can show a significant increase in skin stiffness [107]. Described signs of activity, which can be seen at the inflammatory stage, include a loss of the definition of the dermal-hypodermal border, an increase of the echogenicity of the hypodermis, and an increased dermal and/or hypodermal blood flow [109]. The combined use of elastography and color Doppler ultrasonography [107] or the use of software that allows the quantification

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Fig. 23.20  Segmental stiff skin syndrome. (a) Clinical picture shows a peau d’orange appearance on the inner left thigh of a 4-year-old girl. (b) HFUS (18 MHz) shows a thickening of the dermis and of the fascia when compar-

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of the sonographic echogenicity [106] may improve the detection of activity and fibrosis. However, more multicentric reports on the use of cutaneous elastography are needed.

Segmental Stiff Skin Syndrome Stiff skin syndrome (SSS), also known as congenital fascial dystrophy, is a rare genetic connective tissue disease resulting from mutations in the fibrillin-1 (FBN1) gene with altered activation and signaling of transforming growth factor beta (TGF-β) and subsequent promotion of profibrotic activity. It is characterized by progressive, noninflammatory fibrosis of the skin that may result in limitation of joint mobility. Segmental stiff skin syndrome (SSSS) has been described as a distinct clinical variant, likely resulting from a mosaic mutation in FBN1. Unlike the widespread version, SSSS has a later onset and a better prognosis. It usually starts during childhood or early adolescence as a progressive unilateral hardening of the skin, involving, most commonly, the shoulder or pelvic girdle and the lower limbs. It typically presents with a peau d’orange appearance, preserved or increased hair follicles, and no pigmentary changes (Fig. 23.20a). Decreased joint mobility and leg length discrepancy can be accompanying manifestations. It is often mistaken with linear morphea or eosinophilic fasciitis [110].

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ing the affected area (left) with the contralateral homologous healthy skin (right). Reuse permitted by Wiley [110, 235]

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Histopathologically, it appears with an increased number of thickened collagen bundles in the reticular dermis, extending towards the hypodermis and entrapping adipocytes, increased dermal mucin, and lack of inflammatory infiltrate [110, 111]. Sonographically, when comparing with the homologous contralateral healthy skin, SSSS will appear with an increased thickness of the dermis, with preserved or decreased echogenicity and preserved hair follicles; a hypodermis with normal thickness and echostructure, but prominent fibrous septae; and a significant increase in the thickness of the fascia (Fig.  23.20b). The decreased dermal echogenicity may reflect the presence of mucin. The increased thickening of the fascia may explain the peau d’orange appearance. Color Doppler mode does not show increased blood flow in the region [110, 112].

Vascular Lesions The information included in this section is based on the latest update in 2018 of the ISSVA classification of vascular anomalies.

Tumors  obular Capillary Hemangioma L (Pyogenic or Telangiectatic Granuloma) Lobular capillary hemangioma, also known as pyogenic or telangiectatic granuloma, is a common benign vascular tumor, which usually develops in the skin or mucosae, but can occur intravenously or subcutaneously and in the gastrointestinal tract. Its pathogenesis is not fully understood, but it has been associated with trauma, pregnancy, immunosuppression, and some medications (retinoids, oral contraceptives, antiretrovirals, and targeted oncological therapies). It is more frequent in children, adolescents, and pregnant women and tends to appear on the fingers and toes, face, and oral or nasal mucosae. It usually presents as a rapidly growing, friable, bright-red papule or nodule, often pedunculated [113].

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Histopathologically, it is composed of endothelial cells forming capillaries, which are characteristically arranged in rounded lobules separated by circumferentially oriented fibrous bands. Larger vessels are often seen leading into the lobules or in the matrix between lobules. The overlying epidermis can be thinner and commonly invaginates around the lesion, creating an epithelial collarette [114, 115]. Sonographically, cutaneous lobular capillary hemangioma usually presents as a well-defined, round lesion, with irregular borders and sometimes polypoid and exophytic, predominantly hypoechoic and located in the dermis and/or in the subcutis [14, 116, 117]. The typical lobular architecture may be seen, with echogenic bands likely representing the fibrous septae, dividing hypoechoic lobules [14, 116]. Color Doppler mode shows variable vascularity, often of high density [116, 118], and larger blood vessels, likely corresponding to the feeder vessels, can be detected within the lesion or at the periphery [116, 117, 119].

I nfantile Hemangioma (IH) IH is the most common pediatric vascular tumor. It has a characteristic natural history, which consists of a proliferative and an involutive phase. It usually presents within the first few weeks of life; however, a subtle precursor lesion may be appreciable from birth [120]. Fast growth takes place during the first 3–5 months [121], and most IHs complete the proliferative phase by 9  months [121]. However, growth can extend up to and beyond 36  months, especially in large, segmental, and/or deep IH and in those located on the head and neck [122]. The involutive phase starts at around 1 year of age and usually lasts for [123] 3–9  years [124], leaving residual lesions in up to 69% of the patients [124]. In general, deep IHs may have a slightly later onset and a more prolonged duration of growth [125]. Clinically, the presentation of IH will depend on its depth. Superficial IHs are localized in the superficial dermis and manifest as a bright-red, finely lobulated, strawberry-like plaque (Fig. 23.21a). Deep IHs occupy the deep dermis and/or subcutaneous tissue and present as a skin-­ colored, lightly bluish, or erythematous subcuta-

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Fig. 23.21 IH and spinal pseudo-dermal sinus tract (PDST) in LUMBAR syndrome. (a). Clinical picture shows a bright-red mamillated plaque on the lumbosacral region of a 10-week-old premature twin, corresponding to IH. Notice the small dimple covered by a translucent layer of thinned skin (arrow), clinical manifestation of the spinal PDST. (b) HFUS (18MHZ), B-mode, longitudinal plane, demonstrates a thickening of the dermis. (c) Color Doppler mode, longitudinal plane, demonstrates abundant vascularity, with slow and fast flows, within the dermis.

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(d) HFUS (18 MHz), B-mode, transverse plane, shows the depression corresponding to the dimple and a sinuous hypoechoic tract extending from the dermis (from the dimple) towards the spinal canal, corresponding to the spinal PDST.  Notice the thickened surrounding dermis and the increased echogenicity in the subcutaneous tissue surrounding the tract, corresponding to the IH. (e) Color Doppler mode demonstrates abundant vascularity in the thickened dermis and within and around the tract

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Fig. 23.22  Deep infantile hemangioma (IH). (a) Clinical picture shows a skin-colored soft mass with some subtle superficial telangiectasias on the glabella of a 7-month-­ old twin boy. (b) HFUS (18 MHz), color Doppler mode,

reveals a subfascial hypoechoic well-defined hypervascular lesion with turbulent blood flow. The underlying frontal bone appears undamaged. Reuse permitted by Wiley [131]

neous mass (Figs. 23.22a and 23.23a). Mixed IHs involve both layers of the dermis and, often, the hypodermis and exhibit clinical features of both superficial and deep IHs (Fig. 23.24a) [126]. IH with minimal or absent growth (IH-MAG) constitutes an unusual variant, characterized by little to no proliferation. It is usually present at birth (75%) [127] and manifests as pink and/or whitish/vasoconstricted blotchy patches with

noticeable telangiectasias. At the periphery, proliferative small bright-red papules and a blanching halo can be seen [128, 129] (Fig. 23.25a). Large (>5 cm) segmental IH affecting the face or scalp may be a feature of PHACE syndrome— posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects or aortic coarctation and eye anomalies. Segmental IH affecting the lumbosacral region and perineum of

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Fig. 23.23  Deep infantile hemangioma (IH). (a) Clinical picture shows an erythematous nodule on the inner third of the eyebrow of a 10-week-old boy, which appeared 6  weeks earlier and was growing. (b) HFUS (18  MHz),

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Fig. 23.24 Mixed infantile hemangioma (IH). (a) Clinical picture shows a bluish mass with superficial bright-red papules on the back of a 2-year-old girl. (b) HFUS (18  MHz), B-mode, demonstrates a dermo-­ hypodermal mass with heterogeneous echogenicity.

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color Doppler mode, demonstrates a mostly hypoechoic dermo-hypodermal tumor with abundant internal vascularity, combining slow and fast flows

Notice that the dermal component is hypoechoic while the hypodermal component is mostly hyperechoic. (c) Color Doppler mode reveals abundant vascularity, with fast and slow flows, despite the age of the patient

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Fig. 23.25 Infantile hemangioma with minimal or arrested growth (IH-MAG). (a) Clinical picture shows a blanched patch with superficial coarse telangiectasias and bright-red peripheral papules. (b) HFUS (18 MHz), color Doppler mode, shows small hypoechoic areas in the der-

mis and a patchy increase in the echogenicity of the uppermost hypodermis, with an increased vascularity within the dermo-hypodermal junction, with slow and fast flows

23  Cutaneous Ultrasonography in Pediatric Dermatology

gluteal cleft may be a feature of LUMBAR syndrome—lower body IH, urogenital anomalies, myelopathy, bone deformity, anorectal malformations or arterial anomalies, and renal anomalies [125] (Fig. 23.21). Histopathologically, IH in the proliferative phase is composed of lobular proliferations of capillaries lined by mitotically active, plump endothelium rimmed by pericytes. Involuting IH shows a reduction in vessel number, with a flattened, less mitotically active endothelium and apoptotic debris in a more conspicuous fibrous and fatty stroma [130]. Sonographic features of IH will depend on its stage in natural history. In the proliferative phase, HFUS demonstrates a predominantly hypoechoic, hypervascular tumor (Figs.  23.21, 23.22, and 23.23), while the involution phase is associated with increased echogenicity and decreased blood vessel density and size [131]. Superficial IHs are confined to the dermis and may appear as a well-­ defined thickening and decreased echogenicity of the dermis (Fig. 23.21) or as a nodular hypoechoic lesion. Deep IHs usually exhibit a nodular or mass-like morphology (Figs.  23.22 and 23.23). Corporal deep IH can present as mostly hyperechoic from an early stage. Corporal mixed IHs often appear with a hypoechoic dermal component and a mostly hyperechoic hypodermal component (Fig. 23.24). Blood vessels may be visible

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in grayscale mode and usually correspond to arteries [132]. With regard to the type of vessel, most IHs contain both arterial and venous vessels [8], and the arterial peak systolic blood flow velocity is high, particularly during the proliferative phase [132] (means in different studies oscillate [8] between 21 and 66  cm/s) [133]. Arteriovenous shunts can be seen in about 20% of the cases. Afferent branches from regional arteries are visible in around 15% of IHs and are more frequently found in IH located on the head and neck [8]. IHs with a hypodermal component tend to maintain the vascularity for longer (Fig. 23.24). IH-MAG does not usually show significant changes using HFUS.  Occasionally, an altered echogenicity, with hyperechoic and hypoechoic areas, and an increase in vascular density, with slow and fast flows, may be noticeable in the dermo-hypodermal junction [127] (Fig. 23.25). HFUS plays a role not only in assisting with differential diagnosis, especially when assessing deep IH (Fig.  23.22), but also in IH classification (superficial, mixed, or deep), assisting with the choice of the most adequate treatment. HFUS helps monitoring and deciding the best moment to withdraw therapy [131] and may allow early detection of rebound growth after discontinuation of oral therapy (Fig.  23.26). Involvement of deep structures (muscle, cartilage, gland, orbit, etc.), presence of arteriovenous

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Fig. 23.26 Infantile hemangioma (IH), rebound. (a) Clinical picture shows a subtle bluish discoloration on the tip of the nose of a 3-year-old girl. She had been previously treated for IH on the same location with oral propranolol for 1  year (starting at 3  months of age) with

complete clinical involution. (b) HFUS (22 MHz), color Doppler mode, reveals a high density of blood vessels, with fast and slow flows, representing rebound growth of IH

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Fig. 23.27 Non-involuting congenital hemangioma (NICH). (a) Clinical picture shows a white macule with erythematous areas on the thigh of a 6-year-old girl. The lesion was present at birth and had not changed. (b) HFUS

(18 MHz) image reveals an ill-defined dermal and hypodermal hyperechoic lesion with tubular and round hypoechoic structures, corresponding to blood vessels (arrow). Reuse permitted by Wiley [131]

shunts, large feeding arterial vessels, and significantly high peak systolic velocity in arterial vessels may be sonographic markers of a prolonged proliferative phase [8].

plastic veins, venous lakes, or ectasias and appear as compressible, long, tortuous channels or lacunae with venous flow [139, 141] (Fig. 23.27). The presence of venous lake in RICH detectable by ultrasound has been associated with a greater risk of bleeding, ulceration, and cardiac failure [139]. Echogenically, CH can appear homogeneously [141] or heterogeneously hypoechoic or heterogeneously hyperechoic [131, 135, 138]. Intravascular thrombi [142] and calcifications [138, 139] may be seen within CH and are not expected in IH. Sonographic monitoring of RICH will reveal a significant decrease in internal vascularity within months, a progressive decrease in size, and subtle increase in echogenicity (Fig. 23.28).

 ongenital Hemangioma (CH) C CH is an uncommon vascular benign tumor that, unlike IH, is characteristically present and fully grown at birth, even though postnatal growth of CH has recently been reported [134]. It is classified based on its clinical behavior as rapidly involuting congenital hemangioma (RICH), non-involuting congenital hemangioma (NICH), and partially involuting congenital hemangioma (PICH). It manifests as a well-circumscribed macule, plaque, or nodule, either pink, red, or purple in color or composed of a mixture of blue, white, and red [135, 136]. The presence of thin or coarse telangiectasias on the surface and of a peripheral halo of blanched skin is characteristic [135, 136] (Figs. 23.27a and 23.28a). Histopathologically, it is composed of variably sized capillary lobules with draining arteries associated with extralobular dysplastic veins and lymphatic vessels and surrounded by fibrous tissue [135, 137]. Sonographically, CH shares many sonographic features with IH, including visible blood vessels in gray scale, high density of blood vessels, high arterial peak systolic velocity, and sporadic presence of arteriovenous shunts [118, 131, 135, 138–140]. However, unlike in IH, visible blood vessels in gray scale usually represent dys-

 ufted Angioma (TA) and Kaposiform T Hemangioendothelioma (KHE) TA and KHE are rare vascular tumors that usually present from birth or during infancy or early childhood [143]. Currently, it is widely believed that they represent opposite ends of a spectrum, rather than two separate variants, with TA considered to be more indolent and KHE locally aggressive [144]. Although both can be associated with Kasabach-Merritt phenomenon, a life-­threatening consumptive coagulopathy, the likelihood is higher for KHE [145]. Clinically, TA has been described as a firm, dusky red, violaceous, or brown macule or plaque, often ill-defined or with irregular borders, sometimes associated with pain, edema, hypertrichosis, or hyperhidrosis. It

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Fig. 23.28  Rapidly involuting congenital hemangioma (RICH). (a) Clinical picture shows a congenital bluish mass with erythematous areas and telangiectasias and a peripheral blanched halo on the arm of a 6-week-old infant. (b) HFUS (18  MHz), B-mode, demonstrates a well-defined, mostly hypoechoic dermo-hypodermal tumor. (c) Color Doppler mode reveals abundant vascularity, with slow and fast flows throughout the lesion. (d) Clinical picture of the same lesion at 3  months of age shows a decrease of the bluish color. (e) HFUS (18 MHz),

B-mode, demonstrates a slight reduction in size and increase in echogenicity at 3  months. (f) Color Doppler mode reveals a significant decrease of vascularity, compared to c. (g) Clinical picture of the same lesion at 6 months of age shows a decrease in bluish and red colors and flattening, when compared to previous. (h) HFUS (18  MHz) demonstrates a reduction in size when compared to previous. (i) Color Doppler mode shows almost no blood vessels within the lesion

usually extends slowly and, rarely, experiences spontaneous regression. The most frequent locations are the neck, trunk, and extremities [143, 144, 146]. KHE manifests as a poorly defined, rapidly enlarging, firm, infiltrative, purple plaque or mass, often associated with pain and swelling, most commonly involving the extremities and trunk [143, 145]. Histopathologically, TA is composed of small lobules of tightly packed bland capillaries that are scattered throughout the reticular dermis and superficial subcutis in a so-called cannonball distribution [144]. Crescent-shaped, thin-walled vessels can be seen in the periphery of the lobules

[144]. KHE is composed of dense lobules of spindled endothelial cells with slit-like vascular channels [144], which can also form a “cannonball” distribution and infiltrate deeper into the subcutis and muscle [145, 147]. Dilated lymphatic vessels can be seen in the periphery of the lobules [144]. Sonographic findings are very subtle, and both tumors are difficult to differentiate from adjacent normal tissue. They both display illdefined margins and heterogeneous echogenicity [143]. TA is usually more superficial (