Inoue C, Tamaoka A, Ohkoshi N, Hayashi A, Shoji S
Department of Neurology, University of Tsukuba.
Rinsho Shinkeigaku. 1998 Feb;38(2):142-5.
Livedo vasculitis is characterized by recurrent livedo reticularis of lower extremities and the histopathological findings of segmental hyalinizing vasculitis in the skin. We report a case of a 26-year-old female who manifested mononeuritis multiplex 7 years after the onset of livedo vasculitis. She showed sensori-motor disturbances in the right median and ulnar nerves and sensory deficits of the bilateral peroneal nerves. Sural nerve biopsy revealed a remarkable loss of large and small myelinated fibers and a few vasculitic changes. Steroids therapy was effective for these neurological symptoms. But paroxysmal numbness appeared later recurrently in the regions of affected nerves with painful ulcerations in the right leg. Laboratory tests indicated increased levels of serum thrombin-antithrombin complex (TAT), and antithrombotic drugs (argatroban) remarkably ameliorated the recurrent symptoms and skin lesions. These findings suggest that the pathogenesis of livedo vasculitis might be related to alterations of the blood coagulation system.
青斑样血管炎的特征是下肢反复出现网状青斑以及皮肤节段性透明变性血管炎的组织病理学表现。我们报告一例26岁女性，在青斑样血管炎发病7年后出现多发性单神经炎。她右侧正中神经和尺神经出现感觉运动障碍，双侧腓总神经有感觉缺失。腓肠神经活检显示大小有髓纤维显著减少以及一些血管炎改变。类固醇治疗对这些神经症状有效。但后来受累神经区域反复出现阵发性麻木，右腿出现疼痛性溃疡。实验室检查表明血清凝血酶 - 抗凝血酶复合物（TAT）水平升高，抗血栓药物（阿加曲班）显著改善了反复出现的症状和皮肤病变。这些发现提示青斑样血管炎的发病机制可能与凝血系统改变有关。
