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Cryoglobulinemic Vasculitis in Disguise: Cryofibrinogenemia as Variant of Monoclonal Gammopathy of Renal Significance.
Am J Kidney Dis
March 2024
Internal Medicine/Nephrology, Meander Medical Centre, Amersfoort, The Netherlands.
Article Synopsis
- A 39-year-old woman diagnosed with relapsing nephrotic syndrome was found to have cryofibrinogen and a monoclonal immunoglobulin (M-protein) in her blood, indicating monoclonal gammopathy related to kidney issues.
- Initial immunosuppressive treatment led to temporary improvement, but after her condition relapsed, she underwent more intensive therapy, including stem cell transplantation, which again provided temporary relief.
- Subsequent experiments revealed that the cryoactivity was dependent on the M-protein, suggesting that cryofibrinogenemia is linked to this protein and should be associated with monoclonal gammopathy of renal significance.
Cryofibrinogenemia: What Rheumatologists Should Know.
Curr Rheumatol Rev
August 2022
Hospital Santa Izabel, Praça Almeida Couto 500, Salvador, Bahia, Brazil.
Cryofibrinogenemia refers to the presence of cryofibrinogen in plasma. This protein has the property of precipitating at lower temperatures. Cryofibrinogenemia is a rare disorder, clinically characterized by skin lesions, such as ulcers, necrosis, livedo reticularis, arthralgia, thrombosis, and limb ischemia.
View Article and Find Full Text PDFArticle Synopsis
- Acute Peripheral and/or Cutaneous Ischemic Syndrome (APCIS) refers to limb and skin necrosis that can arise from various underlying health issues or sometimes from unknown causes.
- The condition can be linked to mechanisms such as vessel wall diseases, embolic events, local blood clots, and certain blood disorders, which complicate diagnosis and treatment.
- The text discusses five cases of APCIS, particularly interesting for rheumatologists, and suggests an initial diagnostic approach along with a treatment plan that includes full-dose anticoagulation and immunosuppressive therapy.
Rare cause of bilateral foot gangrene: coexisting essential cryofibrogenaemia and cryoglobulinaemic vasculitis.
BMJ Case Rep
May 2019
Internal Medicine Department, Groupe Hospitalier Sud Ile de France, Melun, France.
Cryofibrinogenaemia is a rare haematological disorder characterised by cold temperature-induced precipitation of plasma proteins causing small-vessel occlusive vascular disorder with a hallmark of skin ulceration. It remains an underdiagnosed entity because of a lack of diagnostic criteria. Cryoglobulinaemia vasculitis is a small-vessel vasculitis involving the skin, the joints, the peripheral nerve system and the kidneys.
View Article and Find Full Text PDFPenicillin-induced Cutaneous Necrotizing Eosinophilic Vasculitis with Cryofibrinogenemia.
Acta Dermatovenerol Croat
March 2019
Professor Miloš Nikolić, MD, PhD, University of Belgrade School of Medicine Clinic of Dermatovenereology, Clinical Center of Serbia, Pasterova 2, 11000 Belgrade, Serbia;
Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy.
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