| Literature DB >> 31138600 |
Nabil Belfeki1, Sarra Abroug1, Alessio Strazzulla1, Sylvain Diamantis1.
Abstract
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. Its association with cryofibrinogenaemia causes more severe phenotype with poor prognosis. We describe the case of a 59-year-old woman presenting with cold-induced extensive bilateral foot gangrene due to coexisting cryofibrinogenaemia and cryoglobulinaemic vasculitis that required bilateral amputation and rituximab perfusions as maintenance therapy. © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.Entities:
Keywords: immunology; malignant and benign haematology
Mesh:
Substances:
Year: 2019 PMID: 31138600 PMCID: PMC6557395 DOI: 10.1136/bcr-2018-228266
Source DB: PubMed Journal: BMJ Case Rep ISSN: 1757-790X