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KMID : 0363220080460081134
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Korean Journal of Dermatology
2008 Volume.46 No. 8 p.1134 ~ p.1137
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A Case of Fatal Wegener’s Granulomatosis without Anti-neutrophil Cytoplasmic Antibody (ANCA)
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Lim Hyun-Jung
Choe Yoon-Seok
Kim Byung-Soo
Lee Seok-Jong
Kim Do-Won
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Abstract
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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.
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KEYWORD
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ANCA, Necrotizing vasculitis, Wegener’s granulomatosis
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