Cutaneous manifestations of childhood Eosinophilic Granulomatosis with Polyangiitis (cEGPA): A case-based review

Abstract

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.

Keywords: Churg-Strauss syndrome; childhood Churg-Strauss syndrome; childhood eosinophilic granulomatosis with polyangiitis; eosinophilic granulomatosis with polyangiitis.