Rivista di formazione e aggiornamento professionale del pediatra e del medico di base, fondata nel 1982. In collaborazione con l'Associazione Culturale Pediatri.
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"Deep purple": un caso di purpura fulminans in asplenia congenita isolata

A case of purpura fulminans in isolated congenital asplenia

Francesca Cattaneo1, Eugenia Mariani1, Alessandra Lazzerotti2
1Scuola di Specializzazione in Pediatria, Università di Milano-Bicocca
2Fondazione IRCCS “San Gerardo dei Tintori”, Monza, Università di Milano-Bicocca

Settembre 2024 | DOI: 10.53126/MEBXXVIIS129

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 infections. 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.

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