Ghislain Louise, Aydin Selda, Marot Liliane, Demoulin Nathalie, Morelle Johann
Clin Nephrol. 2020 Apr;93(4):209-214. doi: 10.5414/CN109748.
Sneddon's syndrome is a rare, noninflammatory thrombotic vasculopathy characterized by the combination of livedo racemosa, recurrent stroke, and histopathological skin lesions of endarteritis obliterans. Although multiorgan involvement suggests its systemic nature, detailed pathological description of affected organs - including the kidney - is exceptional. We report a case of Sneddon's syndrome with chronic kidney disease, associated with features of endarteritis obliterans in the skin and the kidney. The clinical presentation of our patient is compared to previously reported cases of Sneddon's syndrome with biopsy-proven kidney disease. We also discuss the differential diagnosis, pathophysiological mechanisms, relationship with antiphospholipid syndrome, and management of patients with Sneddon's syndrome and kidney disease. This clinical observation supports the systemic nature of Sneddon's syndrome and provides insights into the mechanisms by which this rare but probably underdiagnosed disease alters kidney function. .
斯内登综合征是一种罕见的非炎症性血栓性血管病，其特征为网状青斑、复发性中风以及闭塞性动脉内膜炎的组织病理学皮肤病变。尽管多器官受累提示其具有系统性，但对包括肾脏在内的受累器官进行详细的病理学描述却很罕见。我们报告一例患有慢性肾脏病的斯内登综合征病例，其皮肤和肾脏具有闭塞性动脉内膜炎的特征。将我们患者的临床表现与先前报道的经活检证实患有肾脏疾病的斯内登综合征病例进行了比较。我们还讨论了斯内登综合征合并肾脏疾病患者的鉴别诊断、病理生理机制、与抗磷脂综合征的关系以及治疗。这一临床观察支持了斯内登综合征的系统性，并为这种罕见但可能诊断不足的疾病改变肾功能的机制提供了见解。
