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.
Irvine
A D
AD
Department of Dermatology, Royal Victoria Hospital, Belfast, Northern Ireland.
Bruce
I N
IN
Walsh
M
M
Burrows
D
D
Handley
J
J
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology; D005385:Fingers / Q000098:blood supply; D006801:Humans; D011695:IgA Vasculitis / Q000276:immunology; D007238:Infarction / Q000276:immunology; D008297:Male; D008875:Middle Aged; D017511:Pyoderma Gangrenosum / Q000276:immunology; D012871:Skin Diseases / Q000276:immunology* / Q000473:pathology
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies