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Rare cause of bilateral foot gangrene: coexisting essential cryofibrogenaemia and cryoglobulinaemic vasculitis
Author(s) -
Nabil Belfeki,
Sarra Abroug,
Alessio Strazzulla,
S. Diamantis
Publication year - 2019
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2018-228266
Subject(s) - medicine , gangrene , vasculitis , amputation , rituximab , dermatology , cryoglobulinemia , pathology , surgery , disease , immunology , lymphoma , hepatitis c virus , virus
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.

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