Korean J Dermatol.  2008 Aug;46(8):1134-1137.

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

• Lim HJ
• Choe YS
• Kim BS
• Lee SJ
• Kim DW

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.

Keyword

ANCA; Necrotizing vasculitis; Wegener's granulomatosis

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Arm
Biopsy
Buttocks
Cyclophosphamide
Glycosaminoglycans
Humans
Inflammation
Lung
Male
Middle Aged
Subcutaneous Tissue
Thorax
Vasculitis
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Glycosaminoglycans