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局限性皮肤系统性硬皮病伴坏疽:一例尸检病例。

Limited cutaneous systemic sclerosis with gangrene: an autopsy case.

机构信息

Department of Internal Medicine, Japan Community Healthcare Organization, Isahaya General Hospital, Isahaya, Japan.

Department of Rheumatology, Japan Community Healthcare Organization, Isahaya General Hospital, Isahaya, Japan.

出版信息

Mod Rheumatol Case Rep. 2020 Jul;4(2):243-247. doi: 10.1080/24725625.2019.1702495. Epub 2019 Dec 20.

Abstract

We describe an autopsy case of a 75-year-old female with limited cutaneous systemic sclerosis (lcSSc) and gangrene due to macrovascular involvement. She was diagnosed with lcSSc complicated with pulmonary arterial hypertension and digital ulcers 9 years before admission. She had recurrent and refractory lower limb ulcers (LLUs), and died because of sepsis caused by gangrene infection. Autopsy findings revealed severely thickened arterial walls of the visceral organs, consistent with vascular involvement of SSc. Systemic vascular involvement in lcSSc may progress in patients with LLUs who harbour several risk factors for vascular involvement.

摘要

我们描述了一例 75 岁女性尸检病例,该患者患有局限性皮肤系统性硬化症(lcSSc)和由于大血管受累导致的坏疽。她在入院前 9 年被诊断为 lcSSc 合并肺动脉高压和指端溃疡。她曾反复发作且难治的下肢溃疡(LLUs),并最终因坏疽感染导致的脓毒症而死亡。尸检结果显示,内脏器官的动脉壁严重增厚,符合 SSc 的血管受累表现。在存在多种血管受累危险因素的 LLUs 患者中,lcSSc 的系统性血管受累可能会进展。

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