Neonatal purpura fulminans: A Rare and fatal disorder – A case series from Western India

A rare but potentially fatal thrombotic condition known as neonatal purpura fulminans (NPF) is brought on by a congenital or acquired lack of natural anticoagulant proteins, specifically protein C or protein S. Disseminated intravascular coagulation (DIC), dermal microvascular thrombosis, perivascular haemorrhage, and progressive haemorrhagic skin necrosis are the pathognomonic features of the syndrome. We present a case series of NPFs from our medical college that manifest as widespread purpura and necrotic skin lesions in the early neonatal era. Significantly low protein C levels and signs of DIC, along with other clinical features, were found during 2023– 2024. In view of fulminant sepsis, inotropic and ventilatory support was started in all the cases. Higher antibiotic therapy was given based on culture sensitivity. As blood investigations showed deranged coagulation parameters, platelet concentrates and fresh frozen plasma were administered. Protein C was given to one of the patients. The patients developed refractory septic shock with rapidly progressive purpura and gangrene involving multiple body areas. Although one of the patients showed remarkable improvement, it eventually led to septicaemia and multi-organ failure. The abrupt onset and rapid progression of gangrenous lesions, combined with documented low protein C (and S in one case) levels, were strongly indicative of NPFs. Early recognition of NPFs is critical, as prompt diagnosis and initiation of appropriate therapy, such as replacement of deficient anticoagulant proteins and management of DIC, can be lifesaving. Our case series demonstrates how crucial it is to take protein C shortage into account when newborns exhibit coagulopathy and extensive purpuric rashes.