Dermatopathology: A volume in the Foundations in Diagnostic Pathology Series, Expert Consult - Online and Print PDF
Preview Dermatopathology: A volume in the Foundations in Diagnostic Pathology Series, Expert Consult - Online and Print
Copyright © 2010 by Saunders, an imprint of Elsevier Inc. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publisher. Notice Knowledge and best practice in this field are constantly changing. As new research and experience broaden our knowledge, changes in practice, treatment and drug therapy may become necessary or appropriate. Readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on their own experience and knowledge of the patient, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the Editor assumes any liability for any injury and/or damage to persons or property arising out of or related to any use of the material contained in this book. Library of Congress Cataloging-in-Publication Data Dermatopathology / edited by Klaus J. Busam. – 1st ed. p. ; cm. – ( Foundations in diagnostic pathology) Includes bibliographical references. ISBN 978-0-443-06654-2 1. Skin–Diseases. 2. Skin–Cancer. I. Busam, Klaus J. II. Title. III. Series: Foundations in diagnostic pathology. [DNLM: 1. Skin Diseases–pathology. 2. Skin Neoplasms–pathology. WR 140 D4351705 2010] RL96.D478 2010 616.5¢07–dc22 2009010247 Publishing Director: William R. Schmitt Developmental Editor: Christine Abshire Publishing Services Manager: Anitha Raj Project Manager: Sruthi Viswam Design Direction: Steven Stave Printed in the United States of America Last digit is the print number 9 8 7 6 5 4 3 2 1 This book is dedicated to my wife and children. To Vinita—for your tireless love and support. You put up with a lot! To Jonathan and Sophia—for the joy of your company. Foreword The study and practice of anatomic pathology are both impressive, including many authors who have contrib- exciting and overwhelming. Surgical pathology, with all uted significantly to the dermatopathology literature. of the subspecialties it encompasses, and cytopathology More important, however, the authors are all practic- have become increasingly complex and sophisticated, ing dermatopathologists with an enormous collective and it is not possible for any individual to master the experience in the field. skills and knowledge required to perform all of these As with all of the other editions in the Foundations tasks at the highest level. Simply being able to make a in Diagnostic Pathology series, the information in the correct diagnosis is challenging enough, but the standard current text is presented in a uniform and organized of care has far surpassed merely providing a diagnosis. fashion, covering the essential clinical and pathologic Pathologists are now asked to provide large amounts features of each entity, accompanied by a large number of ancillary information, both diagnostic and prognos- of practical tables and high-quality photomicrographs. tic, often on small amounts of tissue, a task that can be Dermatopathology in particular lends itself to an algo- daunting even to the most experienced pathologist. rithmic approach to diagnosis, on which this book Although large general surgical pathology textbooks consistently capitalizes. When applicable, the authors are useful resources, they by necessity could not possibly integrate modern ancillary diagnostic techniques, includ- cover many of the aspects that pathologists need to know ing immunohistochemistry, immunofluorescence, elec- and include in their reports. Accordingly, the concept tron microscopy, and molecular diagnostics. behind Foundations in Diagnostic Pathology was born. The book is organized into four major sections, This series is designed to cover the major areas of surgi- including histology of the skin, non-neoplastic derma- cal pathology and cytopathology, and each edition in the tologic disorders, and, of course, tumors and pseudotu- series is focused on one major topic. The goal of every mors of the skin and subcutis. The final unique section book in this series is to provide the essential information summarizes the salient features of the pathologic evalu- that any pathologist, whether general or subspecialized, ation of sections derived from Mohs surgery. in training or in practice, will find useful in the evalua- I wish to extend my heartfelt appreciation to Dr. Busam, tion of virtually any type of specimen encountered. who took on this project despite innumerable other Dr. Klaus Busam, a world-renowned dermatopathol- professional and personal responsibilities. As usual he ogist at Memorial Sloan-Kettering Cancer Center, has provided enormous energy and wisdom in completing edited a superb state-of-the-art book covering the essen- this endeavor. I would also like to extend my sincere tial aspects of dermatopathology. This book is geared appreciation to the many authors who have taken the not only to dermatopathologists or those who have a time from their busy lives to provide their expertise. particular interest in this topic, but also to general sur- It is my hope you find this volume of Foundations in gical pathologists who encounter skin specimens as Diagnostic Pathology to be a valuable help in your daily part of their daily practice. The list of contributors is practice of dermatopathology. John R. Goldblum, md vii Preface When I was first approached with the idea of editing Minimizing references to save space for text and illustra- this book, I hesitated, wondering whether it was worth tions seemed justifiable in an era when ready access to publishing another textbook on dermatopathology, a the Internet permits quick retrieval of specialized arti- field with a number of excellent books already avail- cles and new publications are added to the literature at able, including comprehensive reference texts as well as a rapid pace. Controversial issues could not be discussed basic introductions. On the other hand, pathology and in detail, but we tried to offer a balanced view and prac- dermatology residents have been telling me that they tical guide to deal with ambiguity and complexity. miss a textbook that covers more than the very basics, As editor I would like to thank all the contributors but avoids an exhaustive attempt to review all that is who made this book possible. It is the result of team- known in the field, and is compact enough so that they work. I would also like to thank many individuals at can read it from start to finish during their residency Memorial Sloan-Kettering Cancer Center who provided training. Such an introductory to midlevel book is what direct or indirect help for this book, including Jennifer the Foundation in Diagnostic Pathology series attempts Nobrega, Jason Bini, Allyne Manzo, Kin Kong, and to provide. We wanted to offer a no-nonsense practical Drs. Melissa Pulitzer, Ruby Delgado, and Bijal Amin. book with emphasis on the clinical and histologic fea- I am also grateful to Dr. Marc Rosenblum, Chair of Pat h o - tures required to make a diagnosis. In an effort to keep logy, and Dr. Allan Halpern, Chief of Dermatology, for a the book compact, compromises were necessary. A num- supportive professional environment. Special thanks ber of clinical issues or disease entities had to be left out go to members of the Dermatology Service for providing or could be mentioned only briefly. This affected in par- clinical images and/or valuable comments, in particular ticular rare congenital diseases and entities for which Drs. Kishwer Nehal, Patricia Myskowski, and Ashfaq a skin biopsy provides only limited diagnostic value. Marghoob. Finally I want to express my great apprecia- References were cut to a minimum, offering mainly tion for the support and patience I received from the review articles or specialized books for the reader will- publisher, especially the help from William Schmitt, ing to engage in a more in-depth exploration of a subject. Christine Abshire, and Sruthi Viswam. ix Contributors Bijal Amin, MD Maxwell A. Fung, MD Fellow in Dermatopathology Associate Professor of Clinical Dermatology and Pathology Department of Pathology Department of Dermatology Memorial Sloan-Kettering Cancer Center University of California Davis New York, New York Sacramento, California Wilma F. Bergfeld, MD, FAAD John R. Goldblum, MD Co-Director, Dermatopathology Professor of Pathology Departments of Dermatology and Pathology Department of Anatomic Pathology Senior Staff, Department of Dermatology Cleveland Clinic Lerner College of Medicine Cleveland Clinic Chairman, Department of Anatomic Pathology Cleveland, Ohio Cleveland Clinic Cleveland, Ohio Steven D. Billings, MD Associate Professor Jacqueline Granese, MD, FCAP Department of Anatomic Pathology Dermatopathologist Cleveland Clinic Lerner College of Medicine Director of Anatomic and Clinical Pathology Co-director, Dermatopathology Section Departments of Pathology and Dermatology Anatomic Pathology and Dermatology Graves Gilbert Clinic Cleveland Clinic Bowling Green, Kentucky Cleveland, Ohio Henry Haskell, MD J. Andrew Carlson, MD, FRCPC Clinical Assistant Professor Professor Department of Dermatology Department of Pathology University of Oklahoma Albany Medical College Oklahoma City, Oklahoma Albany, New York Pathologist Regional Medical Laboratory Julide Tok Celebi, MD Pathologist Associate Professor of Clinical Dermatology Department of Pathology Department of Dermatology St. John’s Medical Center Columbia University Tulsa, Oklahoma New York, New York Helen M. Chen, MD, PhD Jacqueline M. Junkins-Hopkins, MD Clinical Assistant Professor Associate Professor in Dermatology Department of Dermatology Department of Dermatology University of Illinois and Chicago Johns Hopkins University Chicago, Illinois Director, Division of Dermatopathology Department of Dermatology Loren E. Clarke, MD Johns Hopkins Hospital Assistant Professor Baltimore, Maryland Departments of Pathology and Dermatology Penn State College of Medicine Neil Scott McNutt, MD Pathologist/Dermatologist Senior Research Associate Departments of Pathology and Dermatology Laboratory of Investigative Dermatology The Penn State Milton S. Hershey Medical Center The Rockefeller University Hershey, Pennsylvania Consultant in Pathology Department of Pathology Anca G. Prundeanu Croitoru, MD Memorial Sloan-Kettering Cancer Center Dermatopathologist New York, New York CBLPATH Rye Brook, New York Michael E. Ming, MD, MSCE Assistant Professor Douglas R. Fullen, MD Department of Dermatology Associate Professor University of Pennsylvania Department of Pathology and Dermatology Philadelphia, Pennsylvania University of Michigan Ann Arbor, Michigan xi xii L ist of Contributors Rajmohan Murali, MBBS, FRCPA Richard A Scolyer, BMedSci, MBBS, MD, FRCPA, Clinical Senior Lecturer FRCPath, MIAC Discipline of Pathology Clinical Professor The University of Sydney Discipline of Pathology Staff Specialist The University of Sydney Department of Anatomical Pathology Senior Staff Specialist Royal Prince Alfred Hospital Department of Anatomical Pathology Sydney, NSW, Australia Royal Prince Alfred Hospital Consultant Pathologist Sydney, NSW, Australia Melanoma Institute Australia (incorporating the Sydney Consultant Pathologist Melanoma Unit) Melanoma Institute Australia (incorporating the Sydney North Sydney, NSW, Australia Melanoma Unit) North Sydney, NSW, Australia Patricia L. Myskowski, MD Professor Klaus Sellheyer, MD Department of Dermatology Cleveland Clinic Foundation Weill Cornell Medical College Department of Dermatology Attending Physician, Dermatology Service Cleveland, Ohio Medicine Nelson Dermatopathology Associates Memorial Sloan-Kettering Cancer Center Atlanta, Georgia Attending Physician Christopher R. Shea, MD Dermatology Professor, Department of Medicine; Chief, Section of New York-Presbyterian Hospital Dermatology; Director, Residency and Dermatopathology New York, New York Fellowship Programs, Kishwer S. Nehal, MD Department of Medicine, Section of Dermatology Associate Professor of Dermatology The University of Chicago, The University of Chicago Department of Dermatology Medical Center Weill Cornell Medical College Chicago, Illinois Associate Attending Physician John F. Thompson, MD, FRACS, FACS Dermatology/Medicine Professor of Melanoma and Surgical Oncology Memorial Sloan-Kettering Cancer Center Faculty of Medicine New York, New York The University of Sydney Victor G. Prieto, MD, PhD Consultant Surgeon Royal Prince Alfred and Mater Hospitals Professor Sydney, NSW, Australia Departments of Pathology and Dermatology Executive Director University of Texas M.D. Anderson Cancer Center and Melanoma Institute Australia (incorporating the Sydney Laboratory Medicine Melanoma Unit) Houston, Texas North Sydney, NSW, Australia Melissa Pulitzer, MD Marie Tudisco, Phd, HT(ASCP) Assistant Attending Pathologist Histotechnologist Department of Pathology, Department of Dermatology Memorial Sloan-Kettering Cancer Center Memorial Sloan-Kettering Cancer Center New York, New York New York, New York Marcia Ramos-e-Silva, MD, PhD Ravi Ubriani, MD Associate Professor and Head Assistant Professor of Clinical Dermatology Sector of Dermatology Department of Dermatology Federal University of Rio de Janeiro Columbia University Rio de Janeiro, Brazil New York, New York Franco Rongioletti, MD Angela J. Wyatt, MD Professor of Dermatology Clinical Assistant Professor Section of Dermatology and Dermatopathology Department of Dermatology University of Genoa Baylor College of Medicine Genoa, Italy Physician Dermatology Memorial Hermann Southwest Hospital and St. luke’s Episcopal Hospital Houstan, Texas Table of Contents Part I: Non-neoplastic Skin Disorders Section 1:Inflammatory Reactions Chapter 1. Spongiotic Dermatitis Chapter 2. Psoriasiform Dermatitis Chapter 3. Interface Dermatitis Chapter 4. Perivascular Dermatitis Chapter 5. Nodular and Diffuse Infiltrates Chapter 6. Vesicular Dermatitis Chapter 7. Vasculitis Chapter 8. Panniculitis Section 2: Infections Chapter 9. Bacterial Infections Chapter 10. Fungal Infections Chapter 11. Viral Infections Chapter 12. Other Infections Section 3:Miscellaneous Non-Neoplastic Conditions Chapter 13. Genodermatoses Chapter 14. Disorders of Pigmentation Chapter 15. Deposition Disorders Chapter 16. Alopecia Part II: Cutaneous Neoplasms Chapter 17. Epidermal Tumors Chapter 18. Sebaceous Tumors Chapter 19. Follicular Tumors Chapter 20. Sweat Gland Tumors Chapter 21. Melanocytic Tumors Chapter 22. Soft Tissue Tumors Chapter 23. Hematolymphoid Neoplasms Chapter 24. Metastases <PII>, <AID> i seCtion Histology of tHe skin Henry Haskell Skin consists of epidermis and dermis (Fig. I-1). Beneath esses provide contact points for desmosomes, which are the dermis lies the subcutis (or hypodermis). the ultrastructural basis for the tight binding of kerati- nocytes to one another. These processes may be difficult to visualize in normal skin, but they become more evi- dent by intercellular edema (also known as spongiosis). EpidErmis The stratum granulosum, or granular cell layer, is named for the irregular, darkly basophilic keratohy- alin granules that accumulate in the cytoplasm of The epidermis is a stratified squamous epithelium that the cells in this layer as they flatten and mature. At consists mainly of keratinocytes, with an admixture of the superficial edge of the stratum granulosum, pro- melanocytes, Langerhans, and Merkel cells. Of these, grammed cell death occurs. keratinocytes are by far the most numerous, making up On most parts of the body, the skin possesses a single the bulk of the epidermis and giving it its characteris- layer of dead (but functional) cells, the stratum corneum, tic microscopic appearance. The epidermis is typically consisting mostly of keratin. The cells in this layer are divided into four layers (see Fig. I-1): sometimes called corneocytes, to distinguish them from the living cells in the layers below. The stratum corneum 1. Stratum basale (SB) varies widely in thickness according to site: in glabrous 2. Stratum spinosum (SS) skin, it is only a few cell layers thick, and forms a char- 3. Stratum granulosum (SG) acteristic “basket weave” pattern; whereas at acral sites, 4. Stratum corneum (SC) it is both thicker and more compact (Fig. I-2). Where The stratum basale (or stratum germinativum), also the stratum corneum is particularly thick, another layer, known as the basal cell layer, consists of a single layer the so-called stratum lucidum, may be present between of cuboidal keratinocytes that lie atop the basement it and the stratum granulosum. The stratum lucidum membrane (see the following) and are connected to it differs from the stratum corneum only by a pale eosino- by numerous hemidesmosomes. At this depth, keratino- philic appearance and higher lipid content. Although cytes have abundant eosinophilic cytoplasm and ovoid pyknotic nuclei may appear in the stratum corneum or nuclei. As its name implies, division of keratinocytes stratum lucidum in pathologic processes, in normal skin occurs primarily in the stratum germinativum, although the keratinocytes of both these layers are anucleate. mitotic activity is occasionally seen in the lower part of Keratinocytes stain positively for high molecular the stratum spinosum, in particular the cell layer imme- weight cytokeratins such as 34betaE12, but negatively diately above the stratum basale, the parabasal layer. In for Cam 5.2. the process of self-renewal, these cells gradually ascend into the upper layers and are replaced. Melanocytes The stratum spinosum, also known as the spinous cell layer, is named after the spinous processes (or “prick- les”), which connect the keratinocytes in this layer and Melanocytes are found along the dermoepidermal the stratum basale to one another. These spinous proc- junction as well as within hair follicles (Fig. I-3). 1 2 Dermatopathology They are responsible for the production and secretion SC of melanin pigment. Histologically the cells are char- SG acterized by small, dark, ovoid nuclei and scant, clear Epidermis SS cytoplasm. Depending on anatomic site they number Papillary SB from one per ten to one per five basal keratinocytes, Dermis with higher concentrations on the face and genitalia. Although melanocytes produce melanin, pigment is not normally visible in their cytoplasm, as it is rapidly Reticular secreted through their network of dendritic processes Dermis and taken up by basal keratinocytes, where it is stored and gradually broken down. Although the amount of melanin produced and stored varies between darker- Figure i-1 and lighter-skinned individuals, the number of mel- Normal skin with epidermis, papillary and reticular dermis. the epidermis anocytes does not. consists of stratum basale (SB), stratum spinosum (SS), stratum granulo- Normal intraepidermal melanocytes stain immuno- sum (Sg), and stratum corneum (SC). histochemically best for tyrosinase, Melan-A/Mart-1, and microphthalmia transcription factor. HMB-45 may decorate some normal melanocytes, but it is not a sensi- tive reagent for visualizing normal resting melanocytes. S-100 protein stains normal intraepidermal melano- cytes, but it is neither very sensitive nor specific. It also stains Langerhans cells. langerhans cells The Langerhans cell is a dendritic cell that functions in antigen presentation, and travels between the skin and draining lymph nodes. In routine tissue sections of uninflamed skin, these cells are difficult to iden- tify on hematoxylin and eosin (H&E)–stained sec- tions. They are best seen on immunostains for S-100 protein and/or CD1a, which highlight their charac- Figure i-2 teristic location above the stratum basale (Fig. I-4). Normal acral skin with thick compact stratum corneum and acrosyringeal duct (arrow). Figure i-4 Figure i-3 langerhans cells are immunoreactive for CD1a and recognized as dendritic melanocytes (arrow) are present at the dermoepidermal junction. cells within the spinous cell layer.
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