Eosinophilic granulomatosis with polyangiitis: Cutaneous clinical and histopathologic differential diagnosis

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, but severe systemic vasculitis that can affect skin and other organ systems. Diagnostic criteria have evolved, and many attempts have been made to classify the vasculitides based on clinical and/or histopathologic features, with an aim to develop standardized criteria. According to the EGPA Consensus Task Force recommendations, EGPA is a syndrome of asthma, eosinophilia, pulmonary infiltrates, and extrapulmonary vasculitis (such as cutaneous involvement with purpura). Histopathologic evidence of vasculitis in EGPA may be associated with eosinophilic infiltration and/or perivascular granulomatous inflammation. We review clinicopathologic criteria of this enigmatic vasculopathy.