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病例报告:特发性皮下血栓性血管病。

Case Report: Idiopathic Subcutaneous Thrombotic Vasculopathy.

作者信息

Godbe Kerilyn, Elver Ashlie, Chow Peter, Williams Chris, Fraga Garth, Harris Penelope, Taha Mohammed, Bhavsar Dhaval, Korentager Richard

机构信息

Department of Plastic Surgery, University of Kansas Medical Center, Kansas City, KS, United States.

University of Kansas School of Medicine, Kansas City, KS, United States.

出版信息

Front Med (Lausanne). 2022 Apr 15;9:843793. doi: 10.3389/fmed.2022.843793. eCollection 2022.

Abstract

Lower extremity ulcers have significant morbidity, with treatment determined by the underlying disorder. Reported is a 32-year-old female presenting with small skin nodules and bruises across her legs 4 weeks following her second COVID vaccination. These lesions progressed into large, necrotic ulcers over several months. Initial work-up showed widespread pannicular thrombotic vasculopathy with ischemic skin necrosis. The tissue was negative for calcification on Von Kossa histochemistry, and a working diagnosis of subcutaneous thrombotic vasculopathy was suggested. The ulcers progressed despite treatments with corticosteroids, therapeutic anticoagulation, intravenous immunoglobulin, plasmapheresis, sodium thiosulfate, wound care, and repeat debridement. Later debridement specimens demonstrated rare vascular and pannicular calcifications. This finding supports the hypothesis that subcutaneous thrombotic vasculopathy is a precursor to calciphylaxis, the patient's current working diagnosis. However, based on the patient's entire clinical picture, a definitive diagnosis has yet to be found. This report highlights the challenges of working with rare diseases and the importance of multidisciplinary cooperation.

摘要

下肢溃疡具有显著的发病率,其治疗取决于潜在疾病。本文报告了一名32岁女性,在第二次接种新冠疫苗4周后,双腿出现小的皮肤结节和瘀斑。这些病变在几个月内发展为大的坏死性溃疡。初步检查显示广泛的脂膜炎性血栓性血管病伴缺血性皮肤坏死。Von Kossa组织化学检查显示组织无钙化,提示皮下血栓性血管病的初步诊断。尽管使用了皮质类固醇、治疗性抗凝、静脉注射免疫球蛋白、血浆置换、硫代硫酸钠、伤口护理和重复清创等治疗方法,溃疡仍在进展。后来的清创标本显示罕见的血管和脂膜钙化。这一发现支持了皮下血栓性血管病是钙化防御的前驱病变这一假说,这是患者目前的初步诊断。然而,基于患者的整体临床表现,尚未找到明确诊断。本报告强调了处理罕见疾病的挑战以及多学科合作的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1979/9051404/474ca8d01cd7/fmed-09-843793-g001.jpg

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