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皮肤白细胞碎裂性血管病:术语和发病机制综述。

Livedoid vasculopathy: A review with focus on terminology and pathogenesis.

机构信息

Department of Medicine, Division of Hematology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.

Department of Dermatology, University of North Carolina School of Medicine, Chapel Hill, NC, USA.

出版信息

Vasc Med. 2022 Dec;27(6):593-603. doi: 10.1177/1358863X221130380. Epub 2022 Oct 26.

Abstract

Livedoid vasculopathy (LV) is a rare thrombotic vasculopathy of the dermis characterized by painful, relapsing ulcers over the lower extremities. Diagnosis is challenging due to the overlap in clinical appearance and nomenclature with other skin disorders. Treatment selection is complicated by poor understanding of the pathogenesis of LV and lack of robust clinical trials evaluating therapy efficacy. The terminology and pathophysiology of LV are reviewed here, along with its epidemiology, clinical and histologic features, and treatment options. A diagnostic pathway is suggested to guide providers in evaluating for comorbidities, referring to appropriate specialists, and choosing from the available classes of therapy.

摘要

皮肤白细胞碎裂性血管病(LV)是一种罕见的真皮血栓性血管病,其特征是下肢出现疼痛性、复发性溃疡。由于其临床表现和命名与其他皮肤疾病重叠,因此诊断具有挑战性。由于对 LV 发病机制的了解不足以及缺乏评估治疗效果的稳健临床试验,治疗选择也很复杂。本文回顾了 LV 的术语和病理生理学,以及其流行病学、临床和组织学特征和治疗选择。提出了一种诊断途径,以指导提供者评估合并症、转介给适当的专家,并从可用的治疗类别中进行选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f43c/9732787/42b9039a2206/10.1177_1358863X221130380-fig1.jpg

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