Korean J Nephrol.  2009 Jul;28(4):345-349.

Wegener's Granulomatosis Presenting as Hematuria and Necrotic Papules

• Shin HS

Abstract

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 developspecific 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.

Keyword

Wegener granulomatosis; Vasculitis; Hematuria

MeSH Terms

Cytoplasm
Ear
Erythrocytes
Eye
Glomerulonephritis
Hematuria
Humans
Joints
Lung Diseases
Nose
Pharynx
Proteinuria
Purpura
Skin
Vasculitis
Vasculitis, Leukocytoclastic, Cutaneous
Wegener Granulomatosis
Vasculitis, Leukocytoclastic, Cutaneous