DERMATOLOGICAL PRESENTATION OF DISEASE-ASSOCIATED WITH ANTINEUTROPHILCYTOPLASMIC ANTIBODIES - A REPORT OF 2 CONTRASTING CASES AND A REVIEWOF THE LITERATURE

Antineutrophil cytoplasmic antibody (ANCA)-associated disease (AAD) co nstitutes 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 clinica l disease continuum which ranges from renal-limited disease to a wides pread systemic vasculitis, including Wegener's granulomatosis and micr oscopic polyangiitis, In the latter, circulating ANCA are an aid to di agnosis and also may play a pathogenic part. Two contrasting patients with XAD are described, both of whom presented primary with dermatolog ical features. These included a cutaneous purpuric vasculitis, orogeni tal ulceration, infarction of the fingertip, and pyoderma gangrenosum- like ulceration. These cases will familiarize dermatologists with both the concept and dermatological features of AAD.