A Case of Fatal Wegener's Granulomatosis without Anti-neutrophil Cytoplasmic Antibody (ANCA) / 대한피부과학회지

ABSTRACT

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis belonging to the small- to medium-sized vessel. A 46-year-old male presented with erythematous hard plaque on chest, calf and multiple subcutaneous nodules on buttock, upper arm. Incisional biopsy was done and the histopathologic finding revealed the features of necrotizing vasculitis with focal granulomatous inflammation in subcutaneous tissue. PNS MRI showed abnormal meningeal enhancement and lung HRCT imaging showed mild infiltration. Inspite of severe systemic involvement, serum tests about ANCA showed negative result. He was treated with systemic corticosteroid and cyclophosphamide pulse therapy. Initially his condition showed improvement but later the disease worsened.