Cicatricial Alopecia
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Vera Price Paradi Mirmirani (EDs.) Cicatricial Alopecia An Approach to Diagnosis and Management
Editors Vera Price Professor, Department of Dermatology University of California, San Francisco San Francisco, California 94115 USA pricev@derm.ucsf.edu Paradi Mirmirani Staff Physician Department of Dermatology, University of California, San Francisco San Francisco, California 94115, USA and Department of Dermatology Case Western Reserve University Cleveland, OH USA and Department of Dermatology The Permanente Medical Group, 975 Sereno Drive, Vallejo, California 94589, USA paradi.mirmirani@kp.org ISBN 978-1-4419-8398-5 e-isbn 978-1-4419-8399-2 DOI 10.1007/978-1-4419-8399-2 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2011924224 Springer Science+Business Media, LLC 2011 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com)
Preface There have been several recent reviews published of the primary cicatricial alopecias, so why was this monograph produced? We felt there was a need for a richly-illustrated and practical text that would be a teaching vehicle primarily for dermatology residents and interested dermatology colleagues. We realize that hair loss in general is not a topic on the curriculum of all dermatology departments, and cicatricial alopecia (scarring alopecia) in particular is frequently not covered. At the University of California, San Francisco, we have held grand rounds dedicated entirely to the scarring alopecias. Introductory lectures are given highlighting clinical and histologic aspects, current treatments, and a summary of new research. In the patient viewing session, patients with characteristic features of each of the predominantly lymphocytic and neutrophilic cicatricial alopecias are presented. By immersing residents and colleagues in the topic of cicatricial alopecias, they have been able to envision a more global and systematic view of these disorders. The feedback has been tremendous: I finally got it! In writing this monograph, we sought to replicate such a teaching session, and the information is presented as though the reader was shadowing us in the clinic. Each disease chapter is introduced with a clinical scenario of a patient, along with relevant clues for making the diagnosis. The discussion section follows and includes multiple photographs and take-away pearls to provide practical information needed to diagnose, manage, and counsel the patient. The suggested reading is not meant to be an exhaustive literature review, but rather a list of a few salient references. The voices of the patients can be heard in the chapter written by patients who tell of their frustrations in being undiagnosed by doctor after doctor, and untreated or mistreated for years. Our goal is to put the cicatricial alopecias on the center stage for dermatology residents and colleagues, because dermatologists may be the only physicians who can diagnose and treat these patients. San Francisco, CA, USA Vallejo, CA, USA Vera Price Paradi Mirmirani v
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Acknowledgments We have been privileged to care for and follow many patients with cicatricial alopecia, and are grateful to all of them for giving us the first-hand opportunity to learn about these conditions. Our patients emphasize how much research is needed to increase our understanding and improve our current treatments, and we dedicate this monograph to them. We thank the six patients who shared their personal experiences with cicatricial alopecia in Chapter 10. We are indebted to the Cicatricial Alopecia Research Foundation (CARF) for changing the landscape for these diseases. The Foundation was associated with the production of this monograph and is an indispensable resource for patients and doctors alike. We are grateful to the many CARF volunteers who provided valuable contributions during various phases of this monograph. We also thank Sheila Belkin, CARF s CEO, for coordinating the patient chapter and for her encouragement throughout. We would like to thank Dr. Bruce Wintroub for his ongoing support of our interest in cicatricial alopecia. He has been instrumental in fostering collaborations that have moved our work and knowledge forward. We are grateful to many individuals whose help has been invaluable in creating the monograph. Dr. Tim McCalmont guided us through countless scalp biopsies and wrote the chapter on dermatopathology. He and Dr. Phil LeBoit both continue to enlighten us about the histopathology of cicatricial alopecia. Dr. Aman Samrao worked diligently throughout the entire production, and her assistance was indispensable in handling tables, figures, clinical images, and whatever was asked of her. Dr. Jeffrey Donovan gave valuable editorial input and assisted with assembling the references. Dr. Tim Berger helped to update many concepts and to remove old baggage from this field; we greatly appreciate his interest in clarifying the lexicon. We thank Canfield Scientific for generously giving us the use of their Nikon camera. We are indebted to Dr. Sarah Cipriano and Leslie Chau for their expert technical and administrative support. Finally, we gratefully acknowledge the major contribution of Dr. Pratima Karnik whose molecular studies have pioneered a breakthrough in understanding the crucial role of the sebaceous gland in normal hair growth and in the pathogenesis of cicatricial alopecia. We also wish to acknowledge Dr. Kurt Stenn, who for many prior years had advocated further study of the sebaceous gland and its role in hair growth and hair loss. vii
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Contents 1 Introduction... 1 How Are the Cicatricial Alopecias Classified?... 1 What Are the Demographics of Patients with Cicatricial Alopecia?... 1 Why Is Hair Loss in Cicatricial Alopecia Irreversible?... 5 Suggested Reading... 5 2 Clinical Assessment of the Patient... 7 If Time Is Limited On the Initial Visit, Take the Scalp Biopsy On the First Visit, and Complete the Evaluation at Next Visit... 7 Scalp Biopsy... 7 Evaluation of the Patient... 8 History... 8 Examination... 8 Standardized Patient Flow Chart... 12 Suggested Reading... 13 3 Dermatopathology... 15 Timothy H. McCalmont Lymphocyte-Mediated Primary Cicatricial Alopecias... 17 Neutrophil-Mediated Primary Cicatricial Alopecias... 20 Mixed Cicatricial Alopecia... 21 References... 23 4 Medical Management... 25 Choice of Treatment... 25 Counseling and Setting Expectations... 25 Treatment of Predominantly Lymphocytic Cicatricial Alopecia... 26 Treatment of Predominantly Neutrophilic/Plasmacytic Cicatricial Alopecia... 26 Laboratory Workup and Side Effects of Systemic Drugs... 27 ix
x Contents Confounding Factors in Managing Cicatricial Alopecias... 28 Suggested Reading... 28 5 Mechanisms and Current Research... 31 Overview... 31 Animal Models and the Sebotrophic Hypothesis... 31 Stem Cells and Immunology... 31 Lipid Metabolic Pathways and Peroxisomes... 32 Future Areas of Research and Unanswered Questions/Controversies... 33 Suggested Reading... 34 6 Predominantly Lymphocytic Group... 35 Lichen Planopilaris... 35 Clinical Scenario... 35 Making the Diagnosis... 35 Discussion... 37 Treatment... 38 Suggested Reading... 41 Graham Little Syndrome... 41 Clinical Scenario... 41 Making the Diagnosis... 41 Suggested Reading... 42 Frontal Fibrosing Alopecia... 42 Clinical Scenario... 42 Making the Diagnosis... 42 Discussion... 43 Treatment... 44 Suggested Reading... 46 Pseudopelade (Brocq)... 46 Clinical Scenario... 46 Making the Diagnosis... 47 Discussion... 47 Suggested Reading... 49 Central Centrifugal Cicatricial Alopecia... 50 Clinical Scenario... 50 Making the Diagnosis... 50 Discussion... 51 Treatment... 53 Suggested Reading... 55 Chronic Cutaneous Lupus Erythematosus... 55 Clinical Scenario... 55 Making the Diagnosis... 56 Discussion... 56 Suggested Reading... 58 Keratosis Follicularis Spinulosa Decalvans... 58
Contents xi Clinical Scenario... 58 Making the Diagnosis... 58 Discussion... 58 Suggested Reading... 59 7 Predominantly Neutrophilic Group... 61 Folliculitis Decalvans... 61 Clinical Scenario... 61 Making the Diagnosis... 62 Discussion... 62 Suggested Reading... 65 Tufted Folliculitis... 65 Clinical Scenario... 65 Making the Diagnosis... 66 Discussion... 66 Suggested Reading... 68 8 Mixed Group... 69 Dissecting Cellulitis... 69 Clinical Scenario... 69 Making the Diagnosis... 69 Discussion... 70 Suggested Reading... 72 Folliculitis Keloidalis... 72 Clinical Scenario... 72 Making the Diagnosis... 73 Discussion... 73 Suggested Reading... 74 9 End Stage Nonspecific Group... 75 Clinical Scenario... 75 Making the Diagnosis... 75 History... 75 Exam... 75 Scalp Biopsy... 75 Discussion... 75 Suggested Reading... 77 10 Personal Experiences of Six Patients... 79 Sheila Belkin Understanding and Helping Patients with Cicatricial Alopecia... 79 On Fire for Research and a Cure!... 79 Cicatricial Alopecia Needs You!... 80 Looking in the Mirror... 80
xii Contents My Hair Loss Encounter... 82 Looking for Answers... 83 My CICAL Journal... 84 A Call to Action: When in Doubt, Biopsy... 85 Index... 87
Contributors Vera Price, MD, FRCP(C) Professor, Department of Dermatology, University of California, San Francisco, San Francisco, California 94115, USA pricev@derm.ucsf.edu Paradi Mirmirani, MD Staff Physician Department of Dermatology, University of California, San Francisco, San Francisco, California 94115 USA; Department of Dermatology, Case Western Reserve University, Cleveland, OH, USA; Department of Dermatology, The Permanente Medical Group, 975 Sereno Drive, Vallejo, California 94589, USA paradi.mirmirani@kp.org Timothy H. McCalmont, MD UCSF Dermatopathology Service, University of California, San Francisco, 1701 Divisadero Street, 4th Floor, San Francisco, CA 94115, USA tim.mccalmont@ucsf.edu Sheila Belkin CEO, Cicatricial Alopecia Research Foundation, Los Angeles, CA 90064, USA sheila@carfintl.org xiii
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