1/6. Familial primary cryofibrinogenemia.BACKGROUND: To our best knowledge this is the second case ever described of familial primary cryofibrinogenemia (CFG). patients: A 29-year-old Moroccan female and two of her three children suffered from painful purpura, slow healing small ulcerations and edema of both feet during the winter season. Laboratory investigations revealed the presence of cryofibrinogen in their blood plasma. All three patients were otherwise healthy and no associated disease could be demonstrated. CONCLUSIONS: The diagnosis of CFG has to be considered in patients with livedo reticularis, edema, painful purpura and slow healing ulcera after cold exposure. Cryofibrinogen-precipitates in the blood plasma have to be determined. Because secondary CFG occurs much more frequently than the primary form, it is important to rule out associated diseases through extensive physical examination and laboratory investigations. This communication also stresses the importance of a through family history of patients with CFG. An autosomal dominant mode of inheritance is supposed.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis (Clic here for more details about this article) |
2/6. poems syndrome associated with cryoglobulinemia, lymphoma, multiple seborrheic keratosis, and ichthyosis.The case of a patient with poems syndrome is presented. The characteristic features of the disorder were associated with the apparently unique and undescribed findings of multiple seborrheic keratosis, ichthyosis, livedo reticularis, and vasculitis, combined with IgM/IgG cryoglobulinemia. Analysis of the cryoprecipitate revealed monoclonal IgM, with kappa light chains. Six years later an immunoblastic lymphoma developed. Notably, the paraneoplastic cutaneous signs were present 6 years before the development of the immunoblastic lymphoma.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis (Clic here for more details about this article) |
3/6. Reticulate purpura, cryoglobulinaemia and livedo reticularis.Two patients with type I cryoglobulinaemia are described, both of whom presented with purpura in a reticulate distribution on the legs, resembling the pattern of physiological livedo. It appeared that increased cooling due to sluggish blood flow in areas of the skin corresponding to the blue areas of physiological livedo may have caused the localization of cryoprecipitate at these sites. To investigate the pathogenesis of the net-like pattern of purpura, three subjects with physiological livedo reticularis of the thighs were studied with a laser-Doppler velocimeter. In two subjects, mean blood flux in the blue areas was 21% (P < 0.01) and 26% (P < 0.02) lower than in adjacent white areas, whereas in the third there was no significant difference. The cutaneous features of cryoglobulinaemia, and the mechanism of the reticulate purpura in this condition, are discussed.- - - - - - - - - - ranking = 5keywords = livedo reticularis, reticularis (Clic here for more details about this article) |
4/6. stanozolol causes rapid pain relief and healing of cutaneous ulcers caused by cryofibrinogenemia.BACKGROUND: Cutaneous manifestations of cryofibrinogenemia include purpura, ecchymosis, and ulcerations. The histology of these lesions is characterized by intravascular thrombi. OBJECTIVE: Our purpose was to test the efficacy of stanozolol, a drug capable of fibrinolytic enhancement, in treating cutaneous ulcers caused by cryofibrinogenemia. methods: Eight patients with cutaneous ulcerations from cryofibrinogenemia were treated with stanozolol. plasma cryofibrinogen was measured before and during treatment with stanozolol. Histologic evaluation was also performed before treatment and in selected patients during treatment. RESULTS: After treatment, seven of the eight patients had healing of their ulcers, prompt reduction in their pain, and improvement in livedo reticularis and purpura. Four of the eight patients had no detectable plasma cryofibrinogen after treatment. In addition, dermal intravascular thrombi resolved. stanozolol was well tolerated and had minimal side effects. CONCLUSION: We conclude that stanozolol is a safe and effective treatment of the cutaneous manifestations of cryofibrinogenemia.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis (Clic here for more details about this article) |
5/6. Spontaneous necrosis of the skin associated with cryofibrinogenemia, cryoglobulinemia, and homocystinuria.Cryofibrinogenemia has been associated with a variety of skin manifestations including purpura, livedo reticularis, and ulceration. Our patient, who had undergone axillobifemoral bypass 5 years previously, presented following the spontaneous development of a necrotic wound involving the left groin scar. The location of the wound suggested the possibility of underlying graft infection, but indium-111 white blood cell scan and MRI failed to show any evidence of infection. The patient was initially treated with oral antibiotics and outpatient debridement with no improvement. A more aggressive approach with inpatient operative debridement and intravenous antibiotics produced moderate improvement. Three months later, the patient developed an identical necrotic wound in the right groin and subsequently a third lesion involving a scar distant from any of the patient's grafts. No evidence of active vasculitis was seen on microscopic examination of the excised tissues. Cryoglobulin and cryofibrinogen assays were positive, and urinary and plasma homocysteine levels were elevated. The patient was subsequently treated with stanozolol, a low-methionine diet, and outpatient intravenous antibiotics with rapid improvement of her wounds. In patients with spontaneous ulceration of the extremities, particularly when they do not respond appropriately to standard therapy, the possibility of cryoglobulinemia or cryofibrinogenemia should be considered.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis (Clic here for more details about this article) |
6/6. livedo reticularis and Raynaud's phenomenon associated with cryoglobulinaemia but not related to hepatitis c virus in an hiv-1-positive patient.Symptomatic cryoglobulinaemia is infrequent in hiv-1-infected patients, but a few cases have been described [1-3], occasionally associated with hepatitis c virus (HCV) infection [1, 3]. These cases showed rheumatologic [1] or neurologic manifestations [2, 3], but until now no cutaneous symptoms associated with cryoglobulinaemia in HIV-infected patients have been described. We report what we believe to be the first case of cutaneous, symptomatic cryoglobulinaemia in an hiv-1-positive patient, who, in addition, was HCV-negative.- - - - - - - - - - ranking = 0.89423278784276keywords = reticularis (Clic here for more details about this article) |