A 30-year-old male taxi driver developed extensive purpuric necrotic lesions all over his body. He had a consolidated shadow in the right upper lung field, proteinuria with microscopic hematuria, and granulomatous change in the nasal mucosa. The standard regimen of prednisolone alone or in combination with cyclophosphamide showed only a limited effect on the lesion. However, weekly intravenous injections of a high dose of cyclophosphamide (500 mg/week) in combination with 60 mg/day of prednisolone exhibited dramatical suppression of the disease activity.