심한 피부괴사를 보인 Wegener 육아종증

Abstract

Wegener`s granulomatosis is an uncommon condition characterized by necrotizing vasculitis of unknown cause. The classic clinical triad consists of necrotizing granulomatous inflammation of the respiratory tract, necrotizing glomerulonephiritis, and necrotizing vascultitis affecting predominantly small vessels. The skin manifestations are frequent, and the most common lesion is purpura distributed on the limbs and trunk. However, severe skin involvement is not common. A 20-year old female presented with purpuric macules on the whole body, which became necrotic. The lungs or kidneys were not affected. However, the histopathologic findings of the skin and nasal mucosa revealed the features of necrotizing vasculitis and granulomatous inflammation. Therefore, we diagnosed Wegener`s granulomatosis, which showed unusually severe skin necrosis. She was treated with systemic corticosteroids and cyclophosphamide, but leukopenia, sepsis, and infective endocarditis continuously developed. She died of a cerebral hermorrhage due to overdose of coumadin, which had been used following a cardiac operation for infective endocarditis