Neonatal purpura fulminans

Kienast A, Nowak-Gottl U, Krumpel A, Harms E, Rellensmann G, Kurnik K, Hornig-Franz I

Abstract

Congenital homozygous or compound heterozygous protein C deficiency is a rare autosomal recessive inherited disorder. In most cases it becomes manifest as purpura fulminans in the neonatal period. Patients develop disseminated intravascular coagulation as a consequence of accelerated microvascular thrombogenesis. The characteristic clinical picture and a decreased protein C plasma concentration function as diagnostic criteria. Untreated, the disease takes a lethal course. We report on a newborn with purpura fulminans due to congenital homozygous protein C deficiency.