NECROSE CUTANEE REVELANT UN DEFICIT CONGENITAL EN PROTEINE C : A PROPOS DE 3 CAS

Neonatal skin necrosis in the context of a congenital homozygous protein C deficiency is rare inherited autosomal recessive disorder, it characterized by rapidly extensive necrotic patches occurring few hours after birth newborn who doesnt present any hemodynamic disorder. The diagnosis based on assay activity which collapsed or even undetectable. Early and replacement therapy are mainstays management before onset disseminated intravascular coagulation. We report three cases newborns presenting with DIC course was fatal.