Hidradenitis Suppurativa

Hidradenitis SuppurativaA Diagnostic Atlas

First Edition

EDITED BY Giuseppe Micali, MDChair, Professor of DermatologyDepartment of DermatologyUniversity of Catania, Italy

This edition first published 2017 © 2017 Wiley

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by law. Advice on how to obtain permission to reuse material from this title is available at http://www.wiley.com/go/permissions.

The right of Giuseppe Micali to be identified as the author of this work has been asserted in accordance with law.

Registered Office(s) John Wiley & Sons, Inc., 111 River Street, Hoboken, NJ 07030, USA

John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

Editorial Office The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

For details of our global editorial offices, customer services, and more information about Wiley products visit us at www.wiley.com.

Wiley also publishes its books in a variety of electronic formats and by print-on-demand. Some content that appears in standard print versions of this book may not be available in other formats.

Limit of Liability/Disclaimer of Warranty While the publisher and authors have used their best efforts in preparing this work, they make no representations or warranties with respect to the accuracy or completeness of the contents of this work and specifically disclaim all warranties, including without limitation any implied warranties of merchantability or fitness for a particular purpose. No warranty may be created or extended by sales representatives, written sales materials or promotional statements for this work. The fact that an organization, website, or product is referred to in this work as a citation and/or potential source of further information does not mean that the publisher and authors endorse the information or services the organization, website, or product may provide or recommenda-tions it may make. This work is sold with the understanding that the publisher is not engaged in rendering professional services. The advice and strategies contained herein may not be suitable for your situation. You should consult with a specialist where appropriate. Further, readers should be aware that websites listed in this work may have changed or disappeared between when this work was written and when it is read. Neither the publisher nor authors shall be liable for any loss of profit or any other commercial damages, including but not limited to special, incidental, consequential, or other damages.

Library of Congress Cataloging‐in‐Publication Data

Names: Micali, Giuseppe, editor.Title: Hidradenitis suppurativa : a diagnostic atlas / [edited by] Giuseppe Micali.Other titles: Hidradenitis suppurativa (Micali)Description: First edition. | Hoboken, NJ : Wiley, 2017. | Includes bibliographical references and index.Identifiers: LCCN 2017011126 | ISBN 9781119272953 (pbk.)Subjects: | MESH: Hidradenitis Suppurativa–diagnosis | AtlasesClassification: LCC RL201 | NLM WR 17 | DDC 616.5/2–dc23 LC record available at https://lccn.loc.gov/2017011126

Cover images courtesy of the author Cover design by Wiley

Set in 10/12pt Minion Pro by Aptara Inc., New Delhi, India

Printed in Great Britain by GraphyCems

10 9 8 7 6 5 4 3 2 1

Contents

List of contributors, vii

Foreword, ix

1 Introduction, 1Giuseppe Micali, Maria Rita Nasca, and Maria Letizia Musumeci

2 Epidemiology and pathogenesis, 3Vincenzo Bettoli and Giulia Ruina

2.1 Epidemiology, 32.2 Pathogenesis, 3

2.2.1 Genetic factors, 32.2.2 Environmental factors, 32.2.3 Immunity, 4

3 Clinical features and diagnostic clues, 7Maria Letizia Musumeci, Maria Rita Nasca, Piera Catalfo, Karishma Bhatt, Federica Dall’Oglio, and Giuseppe Micali

3.1 Cutaneous findings, 73.1.1 Typical cutaneous lesions, 133.1.2 Other cutaneous lesions, 25

3.2 Alternative clinical phenotypes, 273.3 Diagnostic clues, 27

4 Histopathology, 29Franco Rongioletti

4.1 Histopathological features, 294.2 Immunohistochemistry, 374.3 Histological differential diagnoses, 37

5 Classification and severity scales, 39Giuseppe Monfrecola and Matteo Megna

6 Correlation between severity and its impact on quality of life, 47Nevena Skroza, Alessandra Mambrin, Nicoletta Bernardini, and Maria Concetta Potenza

6.1 Quality of life questionnaires, 476.1.1 Dermatology Life Quality Index, 476.1.2 Pain Visual Analogue Scale, 496.1.3 Other quality of life questionnaires, 496.1.4 Sexual dysfunction questionnaires, 52

7 Comorbidities and complex syndromes, 55Gabriella Fabbrocini and Valerio De Vita

7.1 Comorbidities, 557.1.1 Metabolic syndrome, 55

v

vi Contents

7.1.2 Inflammatory bowel disease, 587.1.3 Pyoderma gangrenosum, 597.1.4 Acne, 607.1.5 Pilonidal cyst, 607.1.6 Psoriasis, 627.1.7 Other disorders, 62

7.2 Complex syndromes, 637.2.1 PASH, PsAPASH, PAPASH, PASS, and SAPHO syndromes, 637.2.2 Follicular occlusion syndromes, 63

8 Complications, 65Teresa Oranges, Valentina Dini, Andrea Chiricozzi, Salvatore Panduri, and Marco Romanelli

9 Ultrasound imaging, 69Antonio Martorell

9.1 Staging, 749.1.1 Sonographic scoring of HS (SOS-HS), 74

9.2 Evaluation of the inflammatory activity, 769.3 Monitoring, 76

10 Radiological imaging, 79Giuseppe Petrillo, Stefano Palmucci, and Claudia Trombatore

10.1 Magnetic resonance imaging, 7910.2 Computed tomography, 8110.3 Positron emission tomography, 85

11 Skin imaging: dermatoscopy, 87Francesco Lacarrubba, Anna Elisa Verzì, Aurora Tedeschi, Federica Fiorentini, and Giuseppe Micali

12 Differential diagnosis, 91Stefano Veraldi, Elena Guanziroli, and Mauro Barbareschi

13 Conclusions, 101Giuseppe Micali

Index, 103

List of contributors

Mauro Barbareschi, MDDermatology Unit, University of Milan, Italy

Nicoletta Bernardini, MDDermatology Unit “Daniele Innocenzi”, Department of Medical and Surgical Sciences and Biotechnologies, “Sapienza” University of Rome, Polo Pontino, Rome, Italy

Vincenzo Bettoli, MDDepartment of Medical Sciences, Section of Dermatology, University of Ferrara, Italy

Karishma Bhatt, BAUniversity of Illinois at Chicago, Chicago, Illinois, USA

Piera Catalfo, MDDepartment of Dermatology, University of Catania, Italy

Andrea Chiricozzi, MDDepartment of Dermatology, University of Pisa, Italy

Federica Dall’Oglio, MD, PhDDepartment of Dermatology, University of Catania, Italy

Valerio De Vita, MDSection of Dermatology, Department of Clinical Medicine and Surgery, University “Federico II”, Naples, Italy

Valentina Dini, MD, PhDDepartment of Dermatology, University of Pisa, Italy

Gabriella Fabbrocini, MDSection of Dermatology, Department of Clinical Medicine and Surgery, University “Federico II”, Naples, Italy

Federica Fiorentini, MDDepartment of Dermatology, University of Catania, Italy

Elena Guanziroli, MDDermatology Unit, University of Milan, Italy

Gregor Borut Ernst Jemec, MDDepartment of Dermatology, Roskilde Hospital, Health Sciences Faculty, University of Copenhagen, Denmark

Francesco Lacarrubba, MDDepartment of Dermatology, University of Catania, Italy

Alessandra Mambrin, MDDermatology Unit “Daniele Innocenzi”, Department of Medical and Surgical Sciences and Biotechnologies, “Sapienza” University of Rome, Polo Pontino, Rome, Italy

Antonio Martorell, MD, PhDDermatology Department, Hospital of Manises, Valencia, Spain

Matteo Megna, MDSection of Dermatology, Department of Clinical Medicine and Surgery, University “Federico II”, Naples, Italy

Giuseppe Micali, MDDepartment of Dermatology, University of Catania, Italy

Giuseppe Monfrecola, MDSection of Dermatology, Department of Clinical Medicine and Surgery, University “Federico II”, Naples, Italy

Maria Letizia Musumeci, MD, PhDDepartment of Dermatology, University of Catania, Italy

Maria Rita Nasca, MD, PhDDepartment of Dermatology, University of Catania, Italy

Teresa Oranges, MDDepartment of Dermatology, University of Pisa, Italy

Stefano Palmucci, MDRadiodiagnostic and Radiotherapy Unit, University of Catania, Italy

Salvatore Panduri, MDDepartment of Dermatology, University of Pisa, Italy

Giuseppe Petrillo, MDRadiodiagnostic and Radiotherapy Unit, University of Catania, Italy

Maria Concetta Potenza, MDDermatology Unit “Daniele Innocenzi”, Department of Medical and Surgical Sciences and Biotechnologies, “Sapienza” University of Rome, Polo Pontino, Rome, Italy

Marco Romanelli, MD, PhDDepartment of Dermatology, University of Pisa, Italy

Franco Rongioletti, MDUnit of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Italy

vii

viii List of contributors

Giulia Ruina, MDDepartment of Medical Sciences, Section of Dermatology, University of Ferrara, Italy

Nevena Skroza, MDDermatology Unit “Daniele Innocenzi”, Department of Medical and Surgical Sciences and Biotechnologies, “Sapienza” University of Rome, Polo Pontino, Rome, Italy

Aurora Tedeschi, MD, PhDDepartment of Dermatology, University of Catania, Italy

Claudia Trombatore, MDRadiodiagnostic and Radiotherapy Unit, University of Catania, Italy

Stefano Veraldi, MDDermatology Unit, University of Milan, Italy

Anna Elisa Verzì, MDDepartment of Dermatology, University of Catania, Italy

Foreword

Hidradenitis suppurativa is a disease easily recognized by patients, although they are unlikely to use its medical name – rather just calling it “boils.” Boils are not nice. According to the Bible, they were one of the ten plagues in Egypt and part of the trials of Job. They are unlikely to be associated with good things in any culture and are therefore in many ways a unique and iconic disease. Nevertheless, hidradenitis suppurativa was described first in the French medical literature by Dr Velpeau in 1839 and established as a specific diagnosis by Dr Verneuil 15 years later. It has been a challenge to patients, physicians, and surgeons ever since.

Being an iconic disease may sound grand, but it is not. It means that, in addition to the manifold symptoms the patients experience from hidradenitis suppurativa and its many comorbidities, the psychological consequences are often even greater. Many patients suffer a significant psycho-social burden from their disease, which they perceive as unclean and stigmatizing even though they try to keep it as a secret to all but their nearest. This is the first reason why this atlas is needed. The burden of keeping a secret can often be alleviated by bringing it out into the open, and what better way to bring the secrets of hidradenitis suppurativa out into the open than through an atlas compiled and written by lead-ing medical experts in the field.

Secondly, the atlas is likely to increase awareness of the dis-ease among other health care professionals. A recent study found that the diagnostic delay for hidradenitis is (on aver-age) 7 years, that is, it takes the average patient 7 years from the first outbreak of what is so easily recognized as a boil to

the point when the diagnosis is made by a physician. The delay does not vary significantly in many different countries, indicating the global scope of the problem for patients. This strongly suggests that it is generally not a well-recognized disease and it is to be hoped that this practical atlas will make more physicians recognize the disease earlier and therefore provide better treatment and care than most patients experi-ence today.

Finally, the atlas is representative of the growing general interest in this most disabling disease among skin diseases. Whereas the scientific literature is currently not as big as for some of the other “big” skin diseases such as psoriasis or acne, it is growing exponentially over the years, that is, in a self-reinforcing manner, with more knowledge generating more academic questions and subsequent studies. The advent of the first registered medical treatment for hidradenitis sup-purativa in 2016 has undoubtedly been instrumental to the increased interest. For the first time evidence-based therapy has become available for the management of the disease, giv-ing not only hope of bettering the situation for many patients but also giving the encouragement to the scientific commu-nity to develop even more and better therapies in the future. The third reason why the publication of this atlas is so timely is therefore the exponential growth of information on the disease. It is an appropriate moment in time to collect state-of-the-art information and present it in a coherent volume in order to bring everybody entrusted with the treatment of hidradenitis suppurativa to the next level.

Prof. Gregor B.E. JemecCopenhagen, 2017

ix