CUTANEOUS WEGENERS GRANULOMATOSIS - CLINICAL, HISTOPATHOLOGIC, AND IMMUNOPATHOLOGIC FEATURES OF 30 PATIENTS

Background: Wegener's granulomatosis (WG) is a systemic disease charac terized by necrotizing granulomatous inflammation and vasculitis. Its cutaneous manifestations vary. Objective: We reviewed and characterize d the clinical, pathologic, and immunopathologic features of the speci fic cutaneous manifestations of WG and investigated the sensitivity an d the specificity of anti-neutrophilic cytoplasmic antibody (c-ANCA) i n the cutaneous manifestations of this disease. Methods: A retrospecti ve analysis was conducted of 244 cases of WG observed between 1988 and 1992. Results: Skin involvement occurred in 14% of the patients and w as more frequent in generalized WG. Skin lesions may be an early premo nitory sign of renal disease. Necrotizing ulcerations resembling pyode rma gangrenosum were not uncommon. Leukocytoclastic vasculitis was the most common cutaneous pathologic pattern. Findings of c-ANCA were pos itive in 81% of patients with cutaneous WG. Conclusion: Skin involveme nt usually occurred at presentation with generalized disease. c-ANCA i s a valuable adjunct to diagnosis and follow-up of WG.