Neonatal purpura fulminans, usually with onset during the first few days after birth, is a fatal disorder. Evidence to date shows it to be due to a complete deficiency of either protein C or protein S. 1, 2 The newborn with homozygous protein C or protein S deficiency first develops disseminated intravascular coagulation (DIC) and fibrin or platelet thrombi in the small vessels of the skin, followed by necrosis and hemorrhage into the dermis and subcutaneous tissues. 1, 3-6 If untreated, the coagulopathy and the skin lesions worsen and finally result in death. 1

Purpura fulminans is a rapidly spreading lesion of the skin, characterized by thrombosis of the small vessels in the dermis and subdermis, bleeding into the skin, bullae formation in the hemorrhagic areas, absence of bleeding at other sites and no large vessel thrombosis, and rapid death. 7 The term" purpura fulminans" was first described in 1887 and was associated with an acquired condition manifesting after a benign infection. 7-9 These hemorrhagic skin lesions appear after a mild or benign infection (varicella or streptococcus), which" prepares" the skin for the generation of the purpuric lesion. 7-9 Coagulation abnormalities are compatible with DIC, and extensive thrombosis within the venules and capillaries of the skin are consistent pathologic findings. 7-9 These fulminating lesions become necrotic, necessitating major debridement or amputation. The mechanism of purpura fulminans initiation and propagation is not fully understood. 10