Department of Dermatology, Hôpital Tenon, APHP, Paris, France.
Acta Derm Venereol. 2018 Oct 10;98(9):842-847. doi: 10.2340/00015555-2965.
Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopathy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Investigation of thrombophilia was performed. Electromyography was undertaken in the presence of symptoms suggesting peripheral neuropathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demonstrating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immunoglobulins, producing a good response in 6 patients.
皮肤白细胞碎裂性血管病是一种罕见的血栓性皮肤疾病。本观察性研究旨在评估皮肤白细胞碎裂性血管病的临床和生物学特征，以及治疗的疗效。纳入的患者在临床和组织学上均具有典型的皮肤白细胞碎裂性血管病表现。进行了血栓形成倾向的检查。在出现提示周围神经病的症状时，进行了肌电图检查。共纳入 18 名女性和 8 名男性患者，发病时的平均年龄为 35.5 岁。20 名患者存在至少 1 种血栓形成倾向因素。10 名患者存在周围神经病，其中 2 名患者的肌肉活检显示存在特发性血栓闭塞性血管病。低分子肝素抗凝治疗是最常开具的治疗方法，且疗效最佳（14 名患者有效）。8 名患者疾病严重，对抗凝治疗无反应，需要静脉注射免疫球蛋白，其中 6 名患者的反应良好。
