Reactive angioendotheliomatosis as a presenting cutaneous manifestation of Hughes syndrome

Abstract

Background Antiphospholipid antibody syndrome is an autoimmune disorder associated with pregnancy complications, venous and/or arterial thrombosis and the presence of antiphospholipid antibodies. This syndrome is known to present with various cutaneous features, but association with reactive angioendotheliomatosis has been described rarely in the literature. Results A woman in her thirties with a past history of three consecutive abortions developed purpuric, ulcerative plaque over the plantar aspect of the foot. Her biopsy showed marked expansion of dermal vasculature due to intravascular cellular proliferation suggestive of reactive angioendotheliomatosis. The intravascular cells stained positive for CD31. Her blood investigations showed positive lupus anticoagulant, antiphospholipid antibodies and anticardiolipin antibodies, leading to a diagnosis of antiphospholipid syndrome also known as Hughes syndrome. Conclusion We suggest that a hypercoagulable state caused the formation of intravascular thrombi leading to reactive angioendotheliomatosis. We report a case of Hughes syndrome with reactive angioendotheliomatosis as the first clinical cutaneous manifestation and treated satisfactorily with anticoagulants and immunomodulators.