“Deep Purple”: un caso di purpura fulminans in asplenia congenita isolata

Abstract

Asplenic patients are well-known to be at higher risk of infections, which are primarily caused by Streptococcus pneumoniae. Such infections may present with a sudden onset and a fulminant course, potentially leading to septic shock, purpura fulminans and other severe complications. The authors present the case of a 2-year-old child with an undiagnosed asplenia, who developed septic shock caused by S. Pneumoniae, and subsequently, a volvulus due to intestinal malrotation. Purpura fulminans is a rare, life-threatening condition characterised by disseminated intravascular coagulation, extensive tissue thrombosis and haemorrhagic skin necrosis. It is primarily associated with meningococcal or pneumococcal infec-tions. Approximately half of pneumococcal purpura fulminans cases occur in asplenic or hyposplenic patients. Pneumococcal vaccination may reduce the risk of infection in asplenic patients, although it does not entirely eradicate this risk. When encountering a septic patient presenting with purpura fulminans, it is crucial to consider the potential underlying asplenia as part of the differential diagnosis.