혈뇨와 괴사성 구진으로 발현한 베게너 육아종증 1예

Wegener`s granulomatosis (WG) is an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel vasculitis characterized by ear, nose, and throat (ENT) involvement, pulmonary disease, and glomerulonephritis, less commonly, the eyes, joints, skin, neurologic and cardiac tissue. Cutaneous manifestation as an initial presentation is unusual and about 15% of patients with WG will develop-specific skin lesions at some time during the course of the disease. The most common skin lesion specific to WG is palpable purpura with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide array of clinical and histologic features may be seen. Glomerulonephritis eventually develops in about 80% of patients, although only 20% have renal disease when they first present. Clues are proteinuria and active urine sediment containing dysmorphic red blood cells and red blood cell casts. We report a patient with Wegener`s granulomatosis who presents as hematuria and necrotic papules.