Cryofibrinogenemia: report of a case.
Cryofibrinogenemia, a rare disease, is diagnosed by the presence of reversible cryoprecipitate in plasma, which may cause various clinical manifestations of thromboembolic or hemorrhagic disorders. A 66-year-old female patient suffered from recurrent reticulate erythema, purpura and gangrene on the distal portion of the extremities and face during the winter for 5 years. Cryofibrinogenemia was proven with the following evidences: (1) Histopathologic findings revealed many eosinophilic cylinders, about 6-9 microns in diameter, deposited within the lumen of the blood vessels in the dermis and subcutaneous tissue. There was no evidence of vasculitis. These cylinders were fibrinogen in nature, as proven by direct immunofluorescence. (2) Cryofibrinogen was detected in the citrated plasma, which was confirmed by double immunodiffusion, the presence of an IgG-kappa M protein was also revealed by immunofixation. Debridement and skin grafts were performed, and prednisolone combined with cyclophosphamide were given. Skin lesions improved without recurrence, and the "cryocrit" decreased from 13.6% to less than 2% during the follow up. We conclude that if there is any case with the similar cutaneous manifestations, cryofibrinogenemia should be considered.
Department of Dermatology, National Taiwan University Hospital, Taipei, R.O.C.
J S
JS
Bair
Y C
YC
Wu
Y C
YC
Lü
D000368:Aged; D003449:Cryoglobulinemia / Q000097:blood* / Q000473:pathology; D003450:Cryoglobulins / Q000737:chemistry*; D005260:Female; D005340:Fibrinogen / Q000737:chemistry*; D015241:Fibrinogens, Abnormal; D005455:Fluorescent Antibody Technique; D005734:Gangrene / Q000097:blood / Q000209:etiology; D006801:Humans; D005779:Immunodiffusion; D011693:Purpura / Q000097:blood / Q000209:etiology; D012867:Skin / Q000098:blood supply / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology; D013923:Thromboembolism / Q000209:etiology / Q000473:pathology
D003450:Cryoglobulins; D015241:Fibrinogens, Abnormal; C041874:cryofibrinogen; D005340:Fibrinogen