1/5. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. purpura fulminans secondary to xanthomonas maltophilia sepsis in an adult with aplastic anemia.purpura fulminans is a rare disease characterized by purpura ecchymosis, hypotension, and fever associated with disseminated intravascular coagulation. It often begins as a benign infectious process and subsequently progresses to a severe, catastrophic outcome. It is recognized to originate from congenital or acquired protein c deficiency. We present an unusual case of an adult with xanthomonas maltophilia sepsis that subsequently developed into purpura fulminans with involvement of the four extremities. We discuss the importance of the protein C system in coagulation homeostasis and its relationship to purpura fulminans.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. purpura fulminans.purpura fulminans is a rare disease that typically begins as a benign infectious process and subsequently progresses to severe sepsis, hypotension, purpura ecchymosis, and disseminated intravascular coagulation. We present an unusual case of an adult who was seen initially with pneumococcal sepsis that subsequently developed into purpura fulminans with major extremity involvement. A multidisciplinary approach is needed in the treatment of this often catastrophic disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. purpura fulminans in an adult.purpura fulminans is a rare disease of childhood, manifested by massive hemorrhage into the skin, widespread thrombosis of venules and arterioles and a hematologic picture of disseminated intravascular coagulation. An unusual case in a 68-year-old man is presented and the mode of therapy discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. purpura fulminans.purpura fulminans is a rare disease characterized by cutaneous ecchymosis, hypotension and fever associated with disseminated intravascular coagulation. It usually follows a preparatory infectious illness. A case in a 55 year old woman challenges the concept that this disease is limited to childhood. A review of 100 case reports is presented in which the often quoted high mortality of 90 per cent is found to be no longer tenable.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |