Sneddon's syndrome and systemic lupus erythematosus with cerebrovascular disturbances and widespread livedo

A comparative clinical and instrumental analysis of 97 patients with Sneddon's syndrome (SS), a combination of cerebrovascular ischemic disturbances with widespread livedo, and 12 patients with systemic lupus erythematosus (SLE) with the same combination, has been conducted. Despite the presence of similar features related to antiphospholipid syndrome (APS) - cerebrovascular disturbances, livedo, fetal loss, peripheral venous thrombosis, thrombocytopenia, antibodies to phospholipids, etc - there were distinct differences between SS and SLE. In SS, no skin lesions ("butterfly", discoid lupus, photosensibilization) typical for SLE as well as sores of mucous oral cavity, polyarthritis, serosity, diagnostically significant titers of antinuclear factor and antibodies to DNA were observed. SS emerged with livedo (44%), cerebrovascular disturbances (24%) and systemic APS appearances (32%). SLE in 75% cases began with its classical symptoms and in 25% with systemic APS signs and never with livedo or cerebrovascular disturbances. For 10,5 8,0 years, no cases of SS were featured by typical SLE symptoms. Pathomorphological study indicated that SS and SLE were independent diseases. Their similarity was due to development of secondary APS, including cerebrovascular disturbances and livedo, in some patients with SLE.