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血栓闭塞性脉管炎:一种导致可逆性雷诺现象的罕见病因。

Thromboangiitis obliterans: a rare cause of a reversible Raynaud's phenomenon.

作者信息

Noël B, Krayenbühl B, Cerottini J P, Guggisberg D, Buxtorf K, Pires A, Panizzon R G

机构信息

Department of Dermatology, DHURDV, CHUV, Lausanne, Switzerland.

出版信息

Dermatology. 2000;200(4):363-5. doi: 10.1159/000018412.

Abstract

A 25-year-old woman with progressive Raynaud's phenomenon and digital necrosis is presented. Systemic sclerosis and other connective tissue disorders as well as atherosclerosis and arterial emboli were excluded with appropriate laboratory examinations. Arteriography revealed multiple palmar and digital occlusions with corkscrew-shaped vessels. Based on these characteristic arteriographic and clinical findings, the diagnosis of thromboangiitis obliterans was finally retained. With intravenous perfusion of the prostacyclin analogue iloprost (2 ng/kg/min, 6 h daily during 21 days), a complete healing of Raynaud's phenomenon and of the digital necrosis was observed. There was no recurrence during the 1-year follow-up. This observation demonstrates that thromboangiitis obliterans is a potential reversible cause of severe Raynaud's phenomenon in young women even in the absence of lower limb involvement. Early recognition is important to avoid irreversible complications such as loss of digits.

摘要

本文报告了一名25岁患有进行性雷诺现象和指端坏死的女性患者。通过适当的实验室检查排除了系统性硬化症和其他结缔组织疾病以及动脉粥样硬化和动脉栓塞。动脉造影显示多个掌部和指部闭塞,伴有螺旋状血管。基于这些特征性的动脉造影和临床发现,最终确诊为血栓闭塞性脉管炎。通过静脉输注前列环素类似物伊洛前列素(2 ng/kg/分钟,每天6小时,共21天),观察到雷诺现象和指端坏死完全愈合。在1年的随访中没有复发。该观察结果表明,即使在没有下肢受累的情况下,血栓闭塞性脉管炎也是年轻女性严重雷诺现象的潜在可逆病因。早期识别对于避免不可逆并发症如手指缺失很重要。

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