Department of Dermatology, Claude Bernard University, Hôpital de l'Hôtel-Dieu, Lyon, France.
Clin Exp Dermatol. 2009 Dec;34(8):e811-3. doi: 10.1111/j.1365-2230.2009.03541.x. Epub 2009 Sep 28.
Livedoid vasculitis (LV) may be an isolated condition or associated with an underlying systemic disease, including coagulation disorders, periarteritis nodosa and systemic autoimmune diseases. The classic histological features of LV (fibrin deposition on dermal vessels and the absence of important perivascular infiltrate or leucocytoclasia) argue against a primary vasculitis and is more in keeping with a thrombotic process. Factor V Leiden mutations have rarely been reported in association with LV. We report a patient with LV, who had doubly inherited thrombophilia, with heterozygous mutations in the Factor V Leiden (G1691A) and prothrombin (G20210A) genes.
皮肤白细胞破碎性血管炎(LV)可能为一种孤立性疾病,也可能与潜在的系统性疾病相关,包括凝血障碍、结节性多动脉炎和系统性自身免疫性疾病。LV 的典型组织学特征(真皮血管纤维蛋白沉积,无重要的血管周围浸润或白细胞碎裂)提示其并非原发性血管炎,更符合血栓形成过程。FV Leiden 基因突变与 LV 相关的报道罕见。我们报告了一名 LV 患者,其存在双重遗传性血栓形成倾向,FV Leiden(G1691A)和凝血酶原(G20210A)基因杂合突变。