线状硬皮病合并中枢神经系统血管炎

目的 描述线状硬皮病合并神经系统损害病例的临床、影像学和病理学表现，探讨血管病变的病理生理意义。方法 对2例伴有脑损害的线状硬皮病患者进行临床、神经影像学和脑活检病理学评价。结果 1例30岁女性患者出现眼动神经麻痹、性发作和偏瘫，神经影像学发现皮肤病变同侧的颅骨萎缩、脑萎缩、白质病变。1例27岁女性患者出现进行性加重的头痛，影像学发现右侧颞枕部和基底节囊状病变、白质脱髓鞘。二者均存在右额部“类军刀伤”线状硬皮病。脑活检病理学发现：血管壁增厚，纤维素样变性，淋巴细胞浸润，灶状缺血性梗死，白质脱髓鞘。结论 线状硬皮病出现皮肤病变同侧神经系统损害，其机制可能是由于中枢神经系统血管炎导致血液动力学异常所致。

Linear Scleroderma Associated with Central Nervous System Vasculitis

Objective To describe the clinical,neuroimaging and neuropathological findings in patients with linear scleroderma ＂en coup de saber＂ （LScs） and to explore the role of cerebrovascular changes in the pathogenesis of neurological abnormalities. Methods Two cases of LScs with intracranial involvement were evaluated by clinical, neuroimaging and neuropathology. Results One 30-year-old woman developed Oculomotor nerve palsy, seizure and Hemiparesis. Radiographic studies demonstrated ipsilateral skull atrophy, cerebral atrophy, white matter lesions directly subjacent to the area of scleroderma. Another 27-year-old woman had progressive severe headache, neuroimaging revealed cyst-like lesion with white matter demyelination in right tempo-occipital lobe and basal ganglia. Both of them had linear scleroderma ＂en coup de saber＂ involving the right side of the scalp. Brain biopsy showed obvious thickening and fibrinoid degeneration of vessel walls, perivascular lymphocytic infiltration, patchy ischemic infarct and white matter demyelination. Conclusion The neurological abnormalities associated with linear scleroderma ＂en coup de saber＂ were caused by hemodynamic disturbances from intracranial vasculitis.