高度の皮膚,気道病変など多彩な臨床症状を呈したWegener肉芽腫症の1症例

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  • A case of Wegener's granulomatosis with various manifestations.

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A case of Wegener's granulomatosis with various manifestations involving skin and the respiratory tract was described.<br>The patient was a 35 year-old man admitted to our hospital because of hoarseness, exertional dyspnea and generalized cutaneous blisters. Hypoxemia was observed on admission and bronchoscopy revealed marked stenosis from the subglottis to the segmental bronchi bilaterally and numerous granulomatous lesions, mucosal erosions, and ulcerations were observed. The bloody blisters involved not only the skin but also the mucosa. Nasopharyngeal, bronchial and skin biopsy revealed typical findings of Wegener's granulomatosis. Though microscopic hematuria and casts were observed, the renal biopsy was negative. Neurological examination revealed stocking-type hypesthesia in the lower extremities.<br>After treatment with cyclophosphamide and prednisolone the condition improved dramatically, and the respiratory symptoms disappeared within a day.

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