METHODS Mucosal and cutaneous manifestations of Wegener's granulomatosis (WG) are usually described separately. Both frequently occur at any time of the illness. The aim of this work was to analyze, retrospectively, dermatologic symptoms of 75 WG cases encountered from 1973 through 1992. All patients fulfilled the American College of Rheumatology criteria for WG. We compared clinical and histologic findings and looked for a relationship between these manifestations, disease activity, and other symptoms of WG. RESULTS Thirty-five patients had skin or mucosa involvement. Clinical features were palpable purpura (26 cases), oral ulcers (15), skin nodules (six), skin ulcers (five), necrotic papules (five), gingival hyperplasia (three), pustules (two), palpebral xanthoma (two), genital ulcer (one), digital necrosis (one), and livedo reticularis (one). Pathologic findings depended on clinical aspects. Thirty-five involved skin or mucosa biopsy specimens were obtained from 24 patients. Nongranulomatous vasculitis was associated with purpuric lesions. Granulomatous inflammation was associated with nonpurpuric lesions. Dermatologic manifestations were associated with a higher frequency of articular and renal involvement (68% vs 25%; 80% vs 47%, respectively). Except for xanthoma, onset of skin or mucosa lesions indicated active systemic disease. These manifestations responded well to steroids and cyclophosphamide. CONCLUSIONS Various dermatologic manifestations are frequently observed in WG. They have distinctive pathologic features and usually indicate the presence of active systemic disease, especially with kidney and joint involvement.