[Sneddon syndrome].

Abstract

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.