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Cutaneous manifestations of childhood Eosinophilic Granulomatosis with Polyangiitis (cEGPA): A case‐based review
Author(s) -
Bridges Catherine,
Shenk Mary Elizabeth Reed,
Martin Kari,
Launhardt Alison
Publication year - 2020
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.14144
Subject(s) - medicine , histopathology , granulomatosis with polyangiitis , eosinophilic , dermatology , vasculitis , pathology , skin biopsy , biopsy , disease
Background/Objectives This study seeks to better define the clinical presentation and histopathology of cutaneous manifestations in childhood eosinophilic granulomatosis with polyangiitis (cEGPA). Methods Case reports were collected from Ovid Medline Database and PubMed using keyword identifiers from 1946 to 2017. Adult patients ≥ 18 years and cases not diagnosed with EGPA by the author were excluded. Sixty‐five case reports of cEGPA were initially identified. These were reviewed individually, and fifty cases were determined to meet the American College of Rheumatology criteria for EGPA. No case series examining the cutaneous morphology and histopathology were identified. Cutaneous morphology, lesion location, and cutaneous histopathology results were recorded. Results were analyzed using summary statistics. Results Sixty‐four percent (32/50) of cEGPA patients presented with cutaneous manifestations. Twenty‐nine cases provided specific morphological descriptions and lesion location. Common manifestations included purpura (15/29), subcutaneous nodules (8/29), and a macular/papular/maculopapular rash (8/29). However, twelve different cutaneous morphologies were identified in this review. Lesions occurred most commonly on the extremities (26/29). Twenty‐two cases reported corresponding cutaneous histopathology, which revealed extravascular eosinophils (15/22), vasculitis (13/22), and granulomas (5/22). Only one biopsy sample (1/22) had all three classical EGPA characteristics of granulomas, extravascular eosinophils, and vasculitis. Conclusion With nearly two‐thirds of cEGPA patients presenting with cutaneous manifestation, this study highlights the importance of clinical recognition of this disease by dermatologists. While the varied morphology of skin lesions and rarity of this disease makes cEGPA a difficult diagnosis, prompt recognition and treatment will improve outcomes in this patient population.