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Cutaneous manifestations of childhood eosinophilic granulomatosis with polyangiitis (cEGPA): A case‐based review

Pediatric Dermatology Mar 30, 2020

Bridges C, et al. - Researchers sought to better characterize the clinical presentation and histopathology of cutaneous manifestations in childhood eosinophilic granulomatosis with polyangiitis (cEGPA). Ovid Medline Database and PubMed yielded 65 case reports of cEGPA from 1946 to 2017. Individual assessment of these led to identification of 50 cases who meet the American College of Rheumatology criteria for EGPA. Cutaneous manifestations were reported in 64% (32/50) of cEGPA patients. Purpura (15/29), subcutaneous nodules (8/29), and a macular/papular/maculopapular rash (8/29) were identified as the common manifestations. However, this review revealed twelve different cutaneous morphologies. Most commonly, lesions occurred on the extremities (26/29). Corresponding cutaneous histopathology was reported in 22 cases, which revealed extravascular eosinophils (15/22), vasculitis (13/22), and granulomas (5/22). All three classical EGPA characteristics of granulomas, extravascular eosinophils, and vasculitis were evident in only one biopsy sample (1/22). While similar to EGPA in adults, subtle differences are observed in frequency of presentation that could be clinically relevant. The typical features of EGPA was evident in cutaneous biopsy which is relatively easy to perform and less invasive, costly, and risky than biopsy of other organ systems.

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