Cutaneous Manifestations of Infection in the Immunocompromised Host Marc E. Grossman • Lindy P. Fox • Carrie Kovarik Misha Rosenbach Cutaneous Manifestations of Infection in the Immunocompromised Host Second Edition Authors Marc E. Grossman, M.D. Carrie Kovarik, M.D. Professor of Clinical Dermatology Assistant Professor of Dermatology College of Physicians and Surgeons Dermatopathology, and Infectious Diseases Director, Hospital Consultation Service University of Pennsylvania New York Presbyterian Hospital Philadelphia, Pennsylvania, USA Columbia University Medical Center New York, New York, USA Misha Rosenbach, M.D. Assistant Professor of Dermatology Lindy P. Fox, M.D. and Internal Medicine Associate Professor of Clinical Dermatology Director, Dermatology Inpatient Consult Service Director, Hospital Consultation Service University of Pennsylvania University of California, San Francisco Philadelphia, Pennsylvania, USA San Francisco, California, USA ISBN 978-1-4419-1577-1 e-ISBN 978-1-4419-1578-8 DOI 10.1007/978-1-4419-1578-8 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2011933837 Springer Science+Business Media, LLC 2012 All rights reserved. Th is 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 devel- oped is forbidden. Th e use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identifi ed 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. Th e 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 to the Second Edition Immunocompromised patients are often some of the sickest patients in the hospital. Dermatologists who focus on inpatient consultations and caring for hospitalized patients are often faced with the gravely ill, severely immunocompromised patient who presents with cutaneous lesions that are a mystery to the primary team and the other involved subspecialty consultants. More often than not these lesions are a sign of an infection, typically one that is potentially life- threatening. Infections in the immunocompromised host often lead to rapid demise, making early recognition of the infectious process crucial to the patient’s survival. Th e dermatologist plays a central role in identifying the pathogenic organism, implementing the appropriate therapy and prolonging or saving a life. Th at is the crux of this book. We have combed the literature carefully and combined what we have read with our experience in caring for these very sick, complex patients to present the cutaneous manifestations of infection in the immunocompromised host. Th is textbook centralizes the available literature on the cutaneous manifestations of infection in the immunocompromised host. Th e book is a collection of well-documented references (confi rmed by biopsy and culture) on specifi c skin lesions of infection that illustrates cutaneous lesions of routine and rare infectious organisms, demonstrates the evolution of skin lesions over time, – with immune reconstitution or with the recovery of neutrophils – and highlights recognizable patterns of infection and likely causes in diff erent clinical settings (human immunodefi ciency virus/acquired immune defi ciency syndrome [HIV/AIDS] vs. post solid organ transplantation vs. neutropenia post chemotherapy vs. bone marrow recovery post hematopoietic stem cell transplantation). We have given particular attention to the pattern of disease produced by routine and opportu- nistic pathogens to simplify the expanding list of infectious disease possibilities and recognize the most likely organism for a given clinical situation (e.g., fever, pneumonia and rash or fever, men- ingitis and rash). Starting with the skin lesion (e.g., acral hemorrhagic bullae or subcutaneous nodules), we present an evidence-based tiered diff erential diagnosis based on a literature review, well-documented case reports and the probability of a specifi c organism manifesting as a specifi c pattern of infection in the immunocompromised patient. Th is approach to skin lesions in the immunocompromised host has resulted in a unique textbook with bold illustrations that can be used as a bedside guide for diagnosis. Th e text has been thoroughly updated and expanded since the fi rst edition to refl ect emerging trends in infectious organisms that cause disease in each subgroup of immunosuppressed patients. A new chapter discussing the role of viruses in potentiating malignancies in the immunocompro- mised patient has been added. Th e collection of images presented here is a rare and precious anthology gathered from our work in the “hospital trenches.” Th is book is written by dermatologists but intended for all physicians who care for immuno- compromised patients, including, but not limited to, internists, transplant surgeons, infectious disease specialists, pediatricians, rheumatologists, and hematologist-oncologists. It is intended to be a diagnostic tool for the clinician, as well as a teaching syllabus for medical students and house staff . v Preface to the First Edition In 1979, after training in internal medicine at the Hospital of the University of Pennsylvania and in dermatology at Columbia-Presbyterian Medical Center, I began performing an in-hospital dermatology consultation service at Columbia. I relished the challenge of treating the sickest patients with the worst rashes. I was often rewarded for a compulsively complete skin examination by fi nding the subtle skin clue which was the diagnostic solution to an otherwise complex clinical problem. Th e patients I treated were usually members of the population of immunocompromised patients, a population whose numbers have increased logarithmically over the past 16 years because of major advances in cancer chemotherapy, transplantation, treatment of autoimmune diseases and the AIDS epidemic. Th e major problem in these compromised patients was often infection. Th e infl ammatory response to the invasive organism was altered by either the primary disease or its treatment. Th us, routine pathogens presented in disguise, and the patient became a living culture plate for oppor- tunistic microorganisms, some of which had never been previously described as human pathogens. Skin lesions had to be evaluated not by the morphology alone, but by the clinical setting in which they occurred. Th ere were no atlases or textbooks available to help me in these critical situations. Nor were there teachers or specialists to depend on for dermatology knowledge and advice in the acute care hospital setting, although the infectious disease experts or intensive care specialists, Harold C. Neu and Glenda Garvey, were always willing to share their considerable expertise and wisdom with me. To fi nd out more about cutaneous lesions in the immunocompromised host, I (or more often the dermatology resident doing consults with me for the month) had to search the literature for case reports which were scattered in all general and subspecialty medical journals. Similarly, the care of the compromised host has been managed by all types of physicians: surgeons, internists, primary care physicians, oncologists, rheumatologists and specialists in AIDS, trans- plantation, infectious disease and dermatology. Th is book is a fi rst attempt to centralize the information on cutaneous lesions of infection in the immunocompromised host; to collect well-documented references (by biopsy or culture) on specifi c skin lesions of infection; to illustrate cutaneous lesions of routine and rare infectious organisms; to demonstrate the evolution of skin lesions over time or with the recovery of neutrophils; to recognize patterns of infection and likely causes in diff erent clinical settings; and to provide a list of pathogens that may cause a particular skin lesion when the host is immunocompromised. Lastly, and perhaps most importantly, this book will be used to teach. I am fortunate to have studied under some of the best physician-teachers, both as a medical student and house offi cer at the University of Pennsylvania Medical Center and as a dermatology resident at the Columbia- Presbyterian Medical Center. I can only try to do as well as those from whom I have learned. Scarsdale, New York Marc E. Grossman, M.D. vii Acknowledgments for the Second Edition Leonard Harber, M.D., former chairman of the department of dermatology at Columbia Presbyterian Medical Center, gave me my start in 1976 by supporting my idea of a dermatology consultation service in the medical center for Presbyterian and Babies Hospital (later renamed NY Presbyterian Hospital and Children’s Hospital of NY). Perhaps the fi rst inpatient consultation service of its kind in dermatology, it morphed under the equally supportive and encouraging department