In this granulomatous vasculitis of unknown cause, fever, weight loss and fatigue accompany nasorespirat-ory symptoms such as rhinitis, hearing loss or sinusitis. Only half of the patients have skin lesions, usually symmetrical ulcers or papules on the extremities. Other organs can be affected, including the eye, joints, heart, nerves, lung and kidney. Antineutrophil antibodies are present in most cases and are a useful but non-specific diagnostic marker. Cyclophosphamide is the treatment of choice, used alone or with systemic steroids.