Deadly Dermatologic Diseases

Deadly Dermatologic Diseases

Deadly Dermatologic Diseases Clinicopathologic Atlas and Text Second Edition Editors Micha e l B. Morg an Kar a M. Tr app

Editors, MD Assistant Professor Department of Dermatology Case Western Reserve University School of Medicine Cleveland OH Michael B. Morgan, MD Director of Dermatopathology Dermatology Specialists of Florida Panama City FL Staff Dermatopathologist Advanced Dermatology and Cosmetic Surgery Clermont FL Professor of Dermatology Michigan State College of Medicine, MD Associate Professor of Dermatology and Pathology MetroHealth Medical Center Case Western Reserve University School of Medicine Cleveland OH Kar a M. Tr app, BA Georgetown University School of Medicine Washington DC ISBN 978-3-319-31564-5 ISBN 978-3-319-31566-9 (ebook) DOI 10.1007/978-3-319-31566-9 Library of Congress Control Number: 2016950590 Springer International Publishing Switzerland 2016 This work is subject to copyright. All rights are reserved 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, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG Switzerland

Prefa ce Primary dermatologic disease can result in significant morbidity and mortality, and the skin can also serve as a useful reflection of internal disease. This textbook is not meant to list and summarize all conditions of the skin which can result in death. Some are intentionally omitted, including melanoma and squamous cell carcinoma. Attempting to summarize these in a single chapter would be a disservice to the complexities of the subject and the abundant research which is present. We intend to broaden the knowledge of clinical identification, histopathology, and treatment of a group of fascinating, occasionally deadly diseases. We also attempt to clarify the story behind how these entities came to the attention of the medical community. There are slight changes from the original edition of Deadly Dermatologic Diseases. Several additional chapters were added. The format now includes 7 sections and 43 chapters. Each section contains etiologically similar conditions. Chapters includes clinical photographs and histopathologic photomicrographs which attempt to capture the essence of each condition. A summary including history, epidemiology, pathogenesis, clinical findings and histopathology are included, with treatment algorithms when appropriate. Physicians and physician extenders from many disciplines will find this a useful reference, including primary care providers, dermatologists, dermatopathologists, and surgical pathologists. Medical students and residents in the above areas of medicine will also find this a captivating look into the complex world of medical dermatology, and the deadly conditions which we appreciate and despise. D av i d R. Crow e, MD Cleveland, OH, Michael B. Morgan, MD Panama City, FL,, MD Cleveland, OH, Ka ra M. Trapp, MD Washington, DC, v

Contents Preface........................................................ Contributors................................................... v xi Part I Malignant Cutaneous Neoplasms 1 Angiosarcoma....................................... 3 Stephen Suah, Dylan Greeney, Michael B. Morgan 2 Life-threatening Lymphomas and Leukemias with Prominent Cutaneous Involvement...................... 11 Brianna Castillo, Michael B. Morgan,, 3 Langerhans Cell Histiocytosis.......................... 23 4 Leukemia Cutis...................................... 29 5 Mast Cell Disease: Urticaria Pigmentosa................. 35 6 Merkel Cell Carcinoma................................ 41 Katelynn Campbell, Kaylan Pustover, Michael B. Morgan 7 Cutaneous Metastasis................................. 47 Bruce R. Smoller, 8 Paget s Disease....................................... 55 9 Kaposi Sarcoma...................................... 61 Part II Hereditary Cancer-Predisposition Syndromes and Paraneoplastic Disorders 10 Muir-Torre Syndrome................................. 69 11 Birt-Hogg-Dubé Syndrome............................ 75 12 Cowden Syndrome................................... 81 13 Gardner Syndrome................................... 87 vii

viii Contents 14 Multiple Cutaneous Leiomyomas....................... 93 15 Lethal Hereditary Vascular Disorders: Osler- Weber- Rendu Syndrome, Ataxia-Telangiectasia, and Fabry s Disease...... 97 Michael Garone, Michael B. Morgan Part III Cutaneous Manifestations of Systemic Disease 16 Acquired Ichthyosis, Acanthosis Nigricans, and Palmar Hyperkeratosis...................................... 105 Kaylan Pustover, Katelynn Campbell, Michael B. Morgan 17 Amyloidosis: Systemic, Nodular, and Epidermal Derived... 111 Nicole Vesely, Brianna Castillo, Michael B. Morgan 18 Gyrate Erythemas: Erythema Gyratum Repens and Erythema Chronicum Migrans.................... 117 Kaylan Pustover, Katelynn Campbell, Michael B. Morgan 19 Multicentric Reticulohistiocytosis...................... 123 20 Lethal Non-Langerhans Cell Histiocytoses: Necrobiotic Xanthogranuloma and Xanthoma Disseminatum......... 129 Ryan Schuering, Michael Garone, Michael B. Morgan 21 Pancreatic Panniculitis............................... 135 22 Scleromyxedema.................................... 139 23 Necrolytic Migratory Erythema........................ 143 Ryan Schuering, Michael Garone, Michael B. Morgan 24 Pyoderma Gangrenosum............................. 149 Part IV Drug Eruptions 25 Eruptive Xanthoma.................................. 157 Ryan Schuering, Michael B. Morgan 26 Toxic Epidermal Necrolysis........................... 163 27 Drug Reaction with Eosinophilia and Systemic Symptoms................................. 169 28 Acute Generalized Exanthematous Pustulosis: AGEP.................................... 173

Contents ix Part V Infectious Diseases 29 Anthrax........................................... 179 30 Ecthyma Gangrenosum.............................. 185 31 Rocky Mountain Spotted Fever and Rickettsioses......... 189 Michael Garone, Ryan Schuering, Michael B. Morgan 32 Smallpox........................................... 195 Nicole Vesely, Brianna Castillo, Michael B. Morgan 33 Staphylococcal Toxin-Mediated Scalded Skin and Toxic Shock Syndromes.............................. 201 Nicole Vesely, Brianna Castillo, Michael B. Morgan 34 Meningococcemia and Purpura Fulminans.............. 205 Part VI Autoimmune Disease 35 Cutaneous and Systemic Lupus Erythematosus........... 215 36 Dermatomyositis.................................... 223 37 Diffuse Systemic Sclerosis............................. 229 38 Pemphigus Vulgaris and Paraneoplastic Pemphigus....... 235 Jeff Kushner, Michael B. Morgan 39 Relapsing Polychondritis............................. 241 40 Graft-Versus-Host Disease............................ 247 Part VII Vasculitis and Vasculopathic Disease 41 Systemic Vasculitis................................... 253 42 Calciphylaxis (Calcific Uremic Arteriolopathy)........... 259 43 Kawasaki Disease.................................... 265 Index........................................................ 271

Contributors Katelynn Campbell, MD Department of Pathology, University of Arkansas, Little Rock, Arkansas, Brianna Castillo, MD Department of Internal Medicine, University of South Florida College of Medicine, Tampa, FL, Dylan Greeney, MD Department of Dermatology, William Beaumont College of Medicine, Rochester, MI, Dav id R. Crowe, MD Department of Dermatology, MetroHealth Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, Michae l Garone Jr, D O Department of Internal Medicine, Largo Medical Center, American Osteopathic College of Dermatology, Largo, FL, Alexander Herbst, MD Department of Internal Medicine, Florida State University College of Medicine, Tallahassee, FL, Jeff Kushner, DO Saint Joseph Mercy Health System, Howell, MI, Kiran Mian, DO Department of Internal Medicine, Largo Medical Center, St. Joseph, MI, Michae l B. Mor g an, MD Dermatopathology, Dermatology Specialists of Florida, Panama City, FL, Advanced Dermatology and Cosmetic Surgery, Clermont, FL, Dermatology Michigan State College of Medicine, Pathology University of South Florida, Tampa, FL, Kaylan Pustover, DO Saint Joseph Mercy Health System, Howell, MI, Ryan Schuering, DO Department of Internal Medicine, Largo Medical Center, Largo, FL, Br uce R. Smol ler University of Rochester Medical Center, Pathology and Laboratory Medicine, Rochester, NY, Stephen Suah, MD Department of Internal Medicine, University of Florida College of Medicine, Gainesville, FL, Stephen C. S omach, MD Dermatology and Pathology, MetroHealth Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH,, BA Georgetown University School of Medicine, Washington, DC, Nicole Vesely, MD Department of Internal Medicine, University of Florida College of Medicine, Gainesville, FL, xi