Sneddon Syndrome: the difficult diagnosis and the failure in treatment with oral direct anticoagulants

Abstract

Sneddon Syndrome (SS) is an uncommon disease characterized by cerebrovascular events and the presence of Livedo Racemosa, sometimes overlapping with other auto-immune diseases, such as antiphospholipid syndrome. It can present with positive or negative antiphospholipid antibodies. These case reports aim to increase the knowledge of the medical community about this rare condition as well as reinforce the treatment options.

Here we present two cases. The first shows a 35-year-old woman with various thrombotic events since a young age with a late diagnosis of SS. The second shows a 47-year-old woman, already with SS diagnosis, who developed a left jugular vein thrombosis, even being anticoagulated with dabigatran.

With these two cases, we can see how misunderstood and misdiagnosed this condition is, resulting in a very late diagnosis and all the sequels that come from that. Additionally, we confirmed that the oral anticoagulants may not be sufficient in preventing cerebrovascular events.