Epithelioid hemangioendothelioma as a rare cause of blue toe syndrome

Blue toe syndrome (BTS), is a well-known entity of toe gangrene and rest pain secondary to micro emboli lodged within the digital arteries. BTS among young patients should alert physicians to look for causes such as trauma, connective tissue disease, hypercoagulability state, and others. We hereby describe a 32-year-old female with right BTS. A mass obstructing 80% of the right popliteal artery lumen was the source of emboli. The histologic results of the replaced arterial segment revealed a thrombus on top of epithelioid hemangioendothelioma. This is the first description of the association between primary vascular tumor and BTS.