肢端坏疽合并尿崩症1例

Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipidus: a case report

1临床资料 患者,男性,64岁,主因＂多饮、多尿,伴烦渴半年,肢端疼痛、变黑3个月＂于2014年2月17日入院。半年前饮水量和尿量由1~2 L/d增至6~7 L/d,伴烦渴,饮水后可缓解。3个月前出现四肢肢端剧烈疼痛,伴感觉减退,局部皮温降低,此后肢端逐渐变黑,部分有破溃、流脓,双手遇冷水变白变红,无口腔、外阴溃疡,无关节肿痛,无光过敏、皮疹,无咳嗽、咯血。

The male patient reported here presented as gangrene and central diabetes insipidus （CDI）, who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud＇ s phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, 132-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no sufficient evidence to diagnose any specific disease; as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis （SNV）. For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0.4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pituitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor.