Wegener' s Granulomatosis / 대한피부과학회지

ABSTRACT

Wegener's granulomatosis is a systemic necrotizing vasculitis of unknown cause. The disease is characterized by the involvement of the upper airway, the lung, and the kidney. Skin lesions are frequent and the most common lesion is purpura distributed on the limbs and trunk. A 34-year-old female showed recurrent purpuric macules on the both lower extremities and buttocks. The patient. showed nasal septal perforation with saddle nose deformity and C-ANCA positivity. Histopathologic findings of purpuric lesion revealed the features of necrotizing vasculitis. The histologic specimen from the nasal cavity showed chronic inflammation with granuloma formation and kidney showed focal necrotizing glomerulonephritis. Therefore, we treated the patient with prednisolone and cyclophosphamide having diagnosed Wegener's granulomatosis.