A Paediatric Case Of Granulomatosis With Polyangiitis Accompanied With Dorsalis Pedis Artery Occlusion And Prominent Cryofibrinogenaemia

Granulomatosis with polyangiitis (GPA) is characterised by necrotizing angiitis of the arterioles, capillary and venules in the respiratory tract and kidney. GPA patients show other various symptoms and emergent progression, though skin lesions caused by obstruction of the middle arteries is not frequent. GPA is rare in children and little is known about the incidence of GPA in children and adolescents. Cryofibrinogen is cryoprecipitate in the plasma and cryofibrinogenaemia is a rare disorder that may develop skin manifestation after cold exposure. The symptom of cryofibrinogenaemia is usually mild, though it sometimes presents severe manifestations. We will report a first paediatric case who overlapped GPA and cryofibrinogenaemia, also accompanying skin gangrene.