STUDY OF ANTIPHOSPHOLIPID ANTIBODIES IN A PATIENT WITH SNEDDONS SYNDROME AND HER FAMILY

Background Sneddon's syndrome is a disease characterized by livedo ret icularis and cerebrovascular lesions, with a hereditary transmission a nd unknown etiopathogenesis. A number of reports have documented a lin k between antiphospholipid antibodies and Sneddon's syndrome with diff erent results. The present work was designed to sequentially study ant iphospholipid antibodies in a patient with Sneddon's syndrome and her family and their potential role in thrombotic events. We used cardioli pin and a mixture of phospholipids from rabbit brain as antigen for an tiphospholipid assays to determine diagnostic usefulness. Case Descrip tions A patient with Sneddon's syndrome and 12 available family member s belonging to three generations were evaluated to determine the prese nce of antiphospholipid antibodies (lupus anticoagulant and anticardio lipin antibodies) during vascular thrombotic events and asymptomatic p eriods. Conclusions Our results support a temporal relation between th rombotic events in Sneddon's syndrome and lupus anticoagulant; anticar diolipin antibodies remained invariable. Our index case patient and he r father could be diagnosed as having primary antiphospholipid antibod y syndrome. Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. The use of a mixture of phospholipids as antigen could present some advan tages in serological studies performed in antiphospholipid syndromes.