Abstract Background Purpura fulminans (PF) is a dermatological emergency with a high mortality rate, characterized by extensive skin necrosis due to microvascular thrombosis. As a manifestation of systemic coagulopathy, PF is frequently associated with disseminated intravascular coagulation (DIC) and sepsis. Dermatologists play a crucial role in its early recognition and multidisciplinary management. Objectives This review aims to provide a comprehensive overview of PF, including its clinical presentation, classification, underlying pathophysiology, diagnostic workup, and current management strategies. Methods A systematic analysis of existing literature on PF was conducted, focusing on clinical features, histopathology, laboratory investigations, and treatment approaches. Results PF presents with painful, well-demarcated purpuric lesions that evolve into necrotic eschars. The disease is classified into neonatal, post-infectious, and acute infectious subtypes, with acute infectious PF being the most common. Pathophysiology involves acquired or congenital deficiencies in protein C and S, leading to unregulated thrombosis and vascular damage. Diagnosis requires a combination of clinical assessment, coagulation studies, and histopathology. Management includes broad-spectrum antibiotics, coagulation support, protein C replacement (in neonatal cases), therapeutic plasma exchange, and skin-directed therapies. Surgical intervention is often necessary for extensive necrosis. Conclusions Given its life-threatening nature, PF demands heightened awareness in the dermatology community. Early diagnosis and prompt intervention can significantly improve outcomes. Strengthening interdisciplinary collaboration between dermatologists, intensivists, and haematologists is essential for optimizing patient care and reducing mortality associated with PF.
Gafoor et al. (Wed,) studied this question.