Sneddon syndrome with multiple cerebral infarctions 12 years after the onset of livedo vasculitis: a possible involvement of platelet activation.

[sneddon syndrome]

Sneddon syndrome is characterized by livedo reticularis and multiple cerebral infarctions . Skin and central nervous system symptoms usually have a synchronous onset and at times initial symptoms affect one of them , the other lagging several years behind . We here report a patient with Sneddon syndrome who developed multiple cerebral infarctions more than 10 years after the onset of livedo reticularis . While the neurological symptoms were apparent , the patient did not display active skin manifestations . Laboratory findings excluded collagen diseases , antiphospholipid antibody syndrome , and inherited quantitative deficiency of protein C , protein S and antithrombin III . Abnormal findings included extremely elevated levels of beta -thromboglobulin and platelet factor -4 in the blood , although these acute phase markers of thrombosis were examined several years after the onset of cerebral infarctions . Platelet activation may have caused Sneddon syndrome in the present case .