Neonatal purpura fulminans beyond sepsis: Early recognition of underlying inherited thrombophilia—A case series

: Neonatal purpura fulminans (NPF), though rare, is an emerging concern in neonatal intensive care, with potential for serious long-term consequences. Although classically recognized in the setting of severe sepsis, NPF may also occur secondary to underlying inherited thrombophilia. Four neonates with neonatal purpura fulminans were identified from our institutional ‘Stroke & Thrombosis Registry’. Underlying aetiology included severe protein C deficiency (n=2), thrombin defect(n=1), and thrombomodulin (THBD) mutation (n=1). Clinical presentation was heterogeneous and ranged from isolated cutaneous gangrene to shock, seizures and central nervous system thrombosis. All infants had rapidly progressive purpuric or gangrenous skin lesions. Management included repeated fresh frozen plasma transfusions, anticoagulation and supportive care. Despite intensive management, three infants had poor outcomes and died, while the surviving infant had neurodevelopmental delay in follow-up. This case series highlights that neonatal purpura fulminans is not exclusively a sepsis-related disorder. The presence of rapidly progressive purpuric or gangrenous lesions in neonates, particularly in the absence of obvious acquired risk factors, should prompt evaluation for underlying inherited thrombophilia and genetic disorders.