Angioendotheliomatosis is an uncommon disease characterized histologically by proliferation of cells within vascular lumina with secondary intravascular thrombi resulting in obliteration of the involved vessels. While angioendotheliomatosis was initially thought to be a single disease entity, recent studies show that the disease may be divided into benign (reactive) and malignant variants, with the malignant variant representing intravascular malignant lymphoma. The reactive variant is rare, with only 16 cases reported in the literature, and is characterized histologically by proliferating endothelial cells within vessel lumina. In this report we present a case of reactive angioendotheliomatosis and review the literature on this rare entity.
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