Multiple Cutaneous Manifestations in ANCA-Positive Eosinophilic Granulomatosis with Polyangiitis before and after Biologic Therapy: Clinical and Histopathologic Characterization of a Paradigmatic Case.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare immune-mediated vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Having systemic and possibly severe involvement, a prompt recognition of its clinical features is crucial to achieve favorable patient outcomes. Although cutaneous manifestations represent key elements, these still remain poorly characterized. We report a case of ANCA-positive EGPA presenting with palpable purpura, livedo reticularis, and pemphigoid-like lesions that was successfully treated with glucocorticoid therapy and rituximab. This report portrays the evolution of cutaneous lesions in ANCA-positive EGPA and demonstrates how dermatologic signs may represent indicators of active disease, allowing for timely diagnosis and for the monitoring of disease activity during treatment.

• Authors: Giorgia Carnicelli, Alvise Sernicola, Vito Gomes, Giulia Cundari, Stefania Trasarti, Roberta Priori, Teresa Grieco
• Journal: Journal of clinical medicine (J Clin Med) Vol. 11 Issue 24 (Dec 15 2022) ISSN: 2077-0383 [Print] Switzerland
• PMID: 36556045 (Publication Type: Case Reports)