Cryofibrinogenemia is due to the presence of reversibly cold-precipitating
plasma proteins and material, consisting mostly of
fibrinogen,
fibronectin, and
fibrin. This condition can be idiopathic or secondary to
infection, thromboembolic states,
neoplasm, or
connective tissue disease. The characteristic lesions of
cryofibrinogenemia include
purpura and ulcerations. Histologically, the lesions of
cryofibrinogenemia demonstrate
fibrin thrombi within vessels, with no evidence of
vasculitis. Treatment of
cryofibrinogenemia should be directed at the underlying disease process, if one can be found. Other treatments have included the
anabolic steroid stanozolol, which is presently unavailable,
anticoagulants,
immunosuppressive agents,
plasmapheresis, and the combination of
streptokinase and streptodornase. We report a case of a 61-year-old male smoker with a 10-year history of intermittent ulcerations of both legs and feet. Two separate biopsies showed epidermal ulceration and thrombi within superficial dermal vessels without evidence of
vasculitis. These findings, together with the presence of elevated plasma
cryofibrinogen, led to the diagnosis of
cryofibrinogenemia. The patient continued to have ulcerations despite efforts to control his
high blood pressure, cold avoidance, local
wound care, and treatment with
pentoxifylline 800 mg three times daily. However, when
colchicine 0.6 mg twice daily was added to the patient's care, this led to rapid healing of his ulcerations. He has remained
ulcer free for 2 years taking the combination of
colchicine and high-dose
pentoxifylline. Efforts to reduce the dose of these agents have repeatedly led to recurrences, and remission has promptly followed re-establishment of the combination. To our knowledge, this is the first report documenting use of the combination of
colchicine and high-dose
pentoxifylline to successfully treat
ulcers due to
cryofibrinogenemia.