Thrombotic vasculopathy associated with cryofibrinogenemia

Cryofibrinogenemia refers to the presence of cold-precipitable plasma proteins associated with a variety of disorders, including malignancies, inflammatory processes, and thrombo-hemorrhagic phenomena. Few cases of essential or primary cryofibrinogenemia are reported. We report a case in a 48-year-old man of essential cryofibrinogenemia and hemorrhagic necrosis of the ears and of the upper and lower extremities. Histopathologic studies demonstrated extensive eosinophilic thrombi in the dermal vasculature, with minimal inflammation and no vasculitis. Cryofibrinogenemia should be considered in the investigation of patients with otherwise unexplained cold intolerance and dermal thrombosis associated with minimal or no inflammation.