Reactive angioendotheliomatosis in association with the antiphospholipid syndrome

A 50-year-old woman with systemic lupus erythematosus developed extensive n ecrotic skin lesions on her chest and abdomen after the discontinuation of warfarin. The presence of antiphospholipid antibodies suggested a diagnosis of antiphospholipid syndrome. Histopathology from a skin lesion demonstrat ed marked expansion of the dermal microvasculature by intravascular cellula r proliferation and focal thrombosis. The intravascular cells stained posit ive for the endothelial cell markers CD31 and factor VIII-associated antige n confirming reactive angioendotheliomatosis (RAE). This report is the firs t to identify RAE occurring in the context of the antiphospholipid syndrome . We suggest that intravascular endothelial proliferation, in concert with thrombosis, contributed to the angio-occlusive pathology.