Forty-six patients (33 women, 13 men, mean age 40 years) with Sneddon''s syndrome characterized by an ischemic cerebrovascular disease combined with widespread livedo were studied. The clinical picture also included fetal loss (20 of 29 women who had become pregnant), peripheral venous thrombosis (12 patients), ischemic heart disease (18 patients), thrombocytopenia (8 of 40 patients), arterial hypertension (27 patients), headache (38 patients), and epileptic seizures (5 patients), and was similar to that of the antiphospholipid syndrome (APS). In 36 of 46 (78%) patients antiphospholipid antibodies (aPL) were found: anticardiolipin antibodies >5 GPL in 23 of 46 (50%) and lupus anticoagulant in 26 of 43 (61 %) patients. The presence of clinical and immunological manifestations of the APS in 78% of patients in the absence of typical systemic lupus erythematosus features allows one to consider these Sneddon''s syndrome cases as examples of primary APS. The pathogenesis of Sneddon''s syndrome in aPL-negative patients needs to be clarified.

Keywords:
Sneddon's syndrome, Primary antiphospholipid syndrome, Anticardiolipin antibodies, Lupus anticoagulant
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© 1994 S. Karger AG, Basel
1994
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