[Cryofibrinogenemia].

Cryofibrinogenemia (CF) is an unrecognized disorder and is rarely symptomatic. CF represents 10 % of the whole cryoproteins. Mean age at diagnosis is between 50 and 60 years. The skin is the most frequently involved organ, in 80 % of patients at diagnosis. Skin lesions include purpura, livedo and Raynaud's phenomenon. In severe cases, skin ulcerations or necrosis and gangrene may occur. Arterial thrombosis is frequently reported (20 to 40 % of cases). Defect in the fibrinolysis process may lead to the cryofibrinogen accumulation and clotting in small and medium arteries and provide a rationale for the use of fibrinolytic agents at least in the most severe cases. In this article, we review the clinical presentation, pathogenesis and treatment of CF.