Sepsis-Associated Purpura Fulminans International Registry – Europe
Purpura fulminans is the clinical manifestation of disseminated thrombosis in dermal and systemic microcirculation. This rare disease is frequently associated with multiple organ failure and represents a life-threatening condition with mortality exceeding 50 %. Apart from isolated idiopathic cases, deficiency of Protein C, a major intrinsic anti-coagulant, is considered the leading cause of Purpura fulminans. In the vast proportion of cases, the condition has been shown to emerge secondary to acquired Protein C deficiency associated with severe sepsis, mostly of meningococcal or pneumococcal origin.
A consistent therapeutic approach to sepsis-associated Purpura fulminans (SAPF) has not been established yet. With exaggerated pro-coagulant activity being confirmed as the key pathogenic aspect, several treatment modalities aiming at the balance restoration in the coagulation cascade have been considered. Based on the experience gathered in patients with inherited Protein C deficiency, supplementation with Protein C formulations has been used as adjuvant therapy in SAPF.
Most of the information on SAPF incidence and therapy has been obtained before 2004. Meanwhile, lack of comprehensive data poses considerable difficulties with regard to its diagnosis and treatment. SAPF causality might have been substantially altered in the wake of widespread meningococcal vaccination. There are neither evidence-based treatment guidelines nor comparative evaluation of the efficacy of different therapeutic approaches. Therefore there is a pronounced need of systematic data collection and evaluation covering several aspects of SAPF, such as epidemiology, causality, morbidity and therapy.