Tor Shwayder - Longitudinal Observation of Pediatric Dermatology Patients

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Tor Shwayder , Samantha L. Schneider , Devika Icecreamwala and Marla N. Jahnke

Longitudinal Observation of Pediatric Dermatology Patients

Tor Shwayder

Pediatric Dermatology, Henry Ford Hospital, Detroit, MI, USA

Samantha L. Schneider

Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA

Devika Icecreamwala

Icecreamwala Dermatology, Berkeley, CA, USA

Marla N. Jahnke

Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA

ISBN 978-3-319-98100-0 e-ISBN 978-3-319-98101-7

https://doi.org/10.1007/978-3-319-98101-7

Library of Congress Control Number: 2018960271

Springer Nature Switzerland AG 2019

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. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This Springer imprint is published by the registered company Springer Nature Switzerland AG

The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

To my wife, Aime, whose love, brilliance, and calm guidance leads me through all of lifes amazing adventures.

TAS

Introduction from the Senior Author (TAS)

Pediatric dermatology has been my life and passion for more than thirty years. Seems like a long time looking forward, but it has gone by quickly. This book was the brainchild of my (then) fellow Dr. Devika Patel Icecreamwala who recognized in my vast library of images (many thousands) the kernel of a book to pass the accumulated knowledge onto future generations. We have tended to illustrate the unusual cases rather than the mundane. My thanks to Drs. Sam Schneider and Marla Jahnke for their dogged contributions and editorial additions.

Contents

Part IEczematous Eruption of Childhood

Part IIPapulosquamous and Related Disorders

Part IIIHereditary Disorders of Cornification

Part IVDisorders of Sebaceous and Sweat Glands

Part VCutaneous Tumors and Tumor Syndromes

Part VIVascular Disorders of Infancy and Childhood

Part VIIBullous Disorders of Childhood

Part VIIIPhotosensitivity and Photoreactions

Part IXVascular Diseases

Part XCollagen Vascular Disorders

Part I Eczematous Eruption of Childhood

Springer Nature Switzerland AG 2019

Tor Shwayder , Samantha L. Schneider , Devika Icecreamwala and Marla N. Jahnke Longitudinal Observation of Pediatric Dermatology Patients https://doi.org/10.1007/978-3-319-98101-7_1

1. Hyper IgE Syndrome

Tor Shwayder

(1)

Pediatric Dermatology, Henry Ford Hospital, Detroit, MI, USA

(2)

Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA

(3)

Icecreamwala Dermatology, Berkeley, CA, USA

Hyper-immunoglobulin E (IgE) syndrome (HIES) is a rare clinical entity that was first recognized by Davis in 1966 that affects 1:100,000 patients annually [).

Table 1.1

Scoring system for Hyper IgE Syndrome (Job syndrome)

Clinical findings
Highest IgE (IU/mL)	<200	200500	5011000	10012000	>2000
Total skin abscesses/boils	None			>4
Total pneumonias	None				>3
Parenchymal lung abnormalities	None	Bronchiectasis	Pneumatocele
Other serious infection	None	Present
Fatal infection	None	Present
Highest eosinophils/uL	<700	701800	>800
Newborn rash	None	Present
Eczema (worst stage)	None	Mild	Moderate	Severe
Sinusitis/otitis (in worst year)				>6
Candidiasis	None	Oral, vaginal	Fingernail	Systemic
Retained primary teeth	None				>3
Scoliosis (maximum curvature)	<10	10-14	1520	>20
Minimal trauma fractures	None		>2
Hyperextensibility	None	Present
Characteristic face	None	Mild	Present
Increased interalar distance	<1 SD	12 SD	>2 SD
High palate	None	Present
Congenital anomaly	None	Present
Lymphoma	None	Present

HIES is characterized by eosinophilia, eczema, elevated IgE levels, and recurrent cutaneous and pulmonary infections. Early cutaneous manifestations of HIES include eczematous eruptions, recurrent abscesses, and chronic mucocutaneous candidiasis. The abscesses are referred to as cold abscesses because they are non-inflammatory and are typically caused by Staphylococcus aureus [].

The pathogenesis involves the JAK/STAT pathway, which is critical in mediating the inflammatory cascade. Mutations in the STAT3 locus on chromosome 17 have been identified in patients with HIES. Other mutations include DOCK8 and Tyk2 mutations.

The differential diagnosis of elevated serum IgE includes Wiscott-Aldrich, Omenn, atypical complete DiGeorge, Netherton and polyendocrinopathy enteropathy X-linked (IPEX) syndromes as well as immune dysregulation syndromes.

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