Digital Skin Necrosis in Congenital Afibrinogenaemia Associated with Hepatitis C Virus Infection, Mixed Cryoglobulinaemia and Anticardiolipin Antibodies

Céline Girard, Bernard Guillot, Christine BiroN, Thierry Lavabre-bertrand, Robert Navarro, Didier Bessis
2005 Acta Dermato-Venereologica  
Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41-year-old woman who suffered from congenital afibrinogenaemia and hepatitis C viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss
more » ... pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.
doi:10.1080/00015550410001053 pmid:15848993 fatcat:7yjrleebjfg7zjpos5cdr5gqee