Beightler E, Diven D G, Sanchez R L, Solomon A R
Department of Dermatology, University of Texas Medical Branch, Galveston 77550.
J Am Acad Dermatol. 1991 Feb;24(2 Pt 2):342-5. doi: 10.1016/0190-9622(91)70048-7.
Cryofibrinogenemia refers to the presence of cold-precipitable plasma proteins associated with a variety of disorders, including malignancies, inflammatory processes, and thrombohemorrhagic 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.
冷纤维蛋白原血症是指存在与多种疾病相关的冷沉淀血浆蛋白，这些疾病包括恶性肿瘤、炎症过程和血栓出血现象。原发性冷纤维蛋白原血症的病例报道较少。我们报告一例48岁男性原发性冷纤维蛋白原血症患者，其耳部及上下肢出现出血性坏死。组织病理学研究显示真皮血管中有广泛的嗜酸性血栓，炎症轻微且无血管炎。对于不明原因的不耐寒以及伴有轻微炎症或无炎症的皮肤血栓形成患者，在调查时应考虑冷纤维蛋白原血症。
