Richard M A, Grob J J, Durand J M, Noë C, Basseres N, Bonerandi J J
Service de Dermatologie et Vénéréologie, Hôpital Sainte-Marguerite, Marseille.
Ann Dermatol Venereol. 1994;121(4):331-7.
Sneddon's syndrome is a rare disease characterised by cerebrovascular ischaemic attacks and generalised livedo. Since previous observations, other symptoms were described: involvement of heart, of kidney, arterial hypertension, complicated obstetric or gynaecologic history in women. Prognosis is highly variable, depending on extent and speed of progression of cerebrovascular changes, which can lead to severe permanent mental deterioration. In livedo, histopathology shows pathological changes of small to medium-sized dermal arteries in a distinct time sequence: an early phase localized in endothelium followed by a late fibrotic phase. No specific laboratory findings are found. Recently some cases were reported in association with antiphospholipids antibodies. The etiopathogeny of Sneddon's syndrome is still unknown and could result from different processes: progression to an autoimmune disease such as lupus erythematosus, primitive endarteritis obliterans, or a new clinical expression of the antiphospholipid antibodies syndrome. At present, none of the therapeutic modalities provides significant improvement.
斯内登综合征是一种罕见疾病，其特征为脑血管缺血性发作和全身性网状青斑。自以往观察以来，还描述了其他症状：心脏、肾脏受累，动脉高血压，女性有复杂的产科或妇科病史。预后差异很大，取决于脑血管变化的程度和进展速度，这可能导致严重的永久性智力衰退。在网状青斑中，组织病理学显示中小真皮动脉在不同时间序列的病理变化：早期局限于内皮，随后是晚期纤维化阶段。未发现特异性实验室检查结果。最近有一些病例报告与抗磷脂抗体有关。斯内登综合征的病因仍不清楚，可能由不同过程导致：进展为自身免疫性疾病，如红斑狼疮、原发性闭塞性动脉内膜炎，或抗磷脂抗体综合征的一种新的临床表型。目前，没有一种治疗方式能带来显著改善。
