Pole Raymond Garcin, Department of Neurology, Hôpital Sainte-Anne, Paris and Paris Descartes University, Inserm UMR 894, France.
J Neurol Sci. 2011 Jun 15;305(1-2):152-5. doi: 10.1016/j.jns.2011.03.001. Epub 2011 Mar 26.
Susac's syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear, presumably resulting from an autoimmune endotheliopathy. We report the first case of SS with histologically proven skin involvement, in a 24-year-old male who presented a subacute encephalopathy, branch retinal artery occlusions and bilateral hearing loss, two weeks after the onset of a livedo racemosa of the flanks and feet. Skin biopsies revealed a thrombus in several dermal arterioles, endothelial cells swelling and a mild perivascular lymphocytic infiltrate, which correspond to the same histological findings as previously observed in brain but also in muscle biopsies of patients with SS. A complete recovery was achieved in 4 months with corticosteroids. Follow-up MRI showed centro-callosal "holes". Skin involvement in SS has pathological plausibility since serum antibodies directly binding to central nervous system but also to generic endothelium cells have been reported. Our report supports that SS is a systemic disease that could affect other organs in addition to the brain, retina and inner ear. We suggest careful skin examination should be considered in patients with a suspicion of SS.
苏萨克斯综合征（SS）是一种罕见的微血管疾病，影响大脑、视网膜和内耳的小动脉前毛细血管，可能是自身免疫性血管内皮病的结果。我们报告首例经组织学证实皮肤受累的 SS 病例，患者为 24 岁男性，在四肢出现 Racemosa 性游走性红斑两周后，出现亚急性脑病、视网膜分支动脉阻塞和双侧听力损失。皮肤活检显示几个真皮小动脉中有血栓形成，内皮细胞肿胀，轻度血管周围淋巴细胞浸润，与以前观察到的大脑、SS 患者的肌肉活检相同的组织学发现相符。皮质类固醇治疗 4 个月后完全恢复。随访 MRI 显示中心胼胝体“孔”。SS 皮肤受累具有病理上的合理性，因为已经报道了血清抗体直接与中枢神经系统结合，也与普通内皮细胞结合。我们的报告支持 SS 是一种系统性疾病，除了大脑、视网膜和内耳之外，还可能影响其他器官。我们建议对怀疑 SS 的患者应仔细进行皮肤检查。
