A rapidly progressing case of granulomatosis with polyangiitis

Background: Granulomatosis with polyangiitis (GPA) is characterized by a necrotizing granulomatous vasculitis of the upper and lower respiratory tract and kidney along with a mixed vessel vasculitis. Usually a marker of severe disease, cutaneous involvement often presents as palpable purpura but may present as necrotic papulonodules or ulcers. Mucosal involvement may present as ulcers or gingival hyperplasia. Histology reveals a leukocytoclastic vasculitis with or without granulomas. Cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) are diagnostic.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research