Purpura fulminans (PF) is a very rare clinicopathologic skin disorder comprising dermal microvascular
thrombosis associated with perivascular
hemorrhage of multiple origins. It may occur as the presenting symptom of severe congenital deficiency of
protein C (PC) or
protein S (PS) during the newborn period, or later in life following oral
anticoagulant therapy with
vitamin K antagonists, or of
sepsis that may be associated with
disseminated intravascular coagulation. Treatment consists of
anticoagulants and PC concentrates during acute episodes. We report our experience in the diagnosis and management of pediatric PF. The medical records of the 6 children aged 2-16 years (median: 5 years) who presented with PF to our tertiary care center between 1996 and 2013 were studied. The
thrombophilia workup revealed either the presence of congenital homozygous PC deficiency, prothrombotic polymorphisms (
factor V Leiden and FIIG20210A heterozygosity), acquired PC/PS deficiency, or no discernible
thrombophilia. The skin
necrosis resolved following conservative fresh-frozen plasma/
anticoagulant therapy in 2 cases, whereas 3 children required interventional plastic surgery. The sixth case, a 10-year-old child with severe PC deficiency, heterozygous
factor V Leiden, and FIIG20210A, received recombinant activated PC. PF in childhood is rare and has multiple etiologies. Understanding of the variable pathogenesis and risk factors will facilitate diagnosis and appropriate clinical management.