Dermatological presentation of disease associated with antineutrophil cytoplasmic antibodies: a report of two contrasting cases and a review of the literature.

Antineutrophil cytoplasmic antibody (ANCA)-associated disease (AAD) constitutes a pathological disease spectrum of a necrotizing vasculitis of small- and medium-sized vessels, extravascular granuloma formation, and necrotizing and crescentic glomerulonephritis, and also a clinical disease continuum which ranges from renal-limited disease to a widespread systemic vasculitis, including Wegener's granulomatosis and microscopic polyangiitis. In the latter, circulating ANCA are an aid to diagnosis and also may play a pathogenic part. Two contrasting patients with AAD are described, both of whom presented primary with dermatological features. These included a cutaneous purpuric vasculitis, orogenital ulceration, infarction of the fingertip, and pyoderma gangrenosum-like ulceration. These cases will familiarize dermatologists with both the concept and dermatological features of AAD.