DP01 (P038) Clinical and histopathological features of purpura fulminans: a retrospective case series

Abstract Purpura fulminans (PF) is a rare but serious condition characterized by purpura and skin necrosis resulting from a hypercoagulable state. This study aimed to evaluate the clinical, histopathological and laboratory features, alongside management approaches and outcomes, in a series of patients diagnosed with PF. By analysing multiple cases, the study seeks to elucidate patterns in presentation and disease progression with the aim to improve diagnostic accuracy and clinical outcomes. We conducted a retrospective analysis of 19 patients presenting with PF at a tertiary care centre. Data were gathered to include clinical notes, laboratory results, histopathology reports and photographic documentation of skin lesions. The cohort spanned all age groups, from infants to older adults. Key variables analysed included clinical provenance, skin findings, haematological parameters, histopathological results, comorbidities, management and outcomes. Statistical analysis was descriptive, focusing on patterns and correlations. The cohort ranged in age from 3 months to 74 years, with 11 male and eight female patients. The primary triggers for PF included severe sepsis secondary to bacterial, viral and fungal infections, frequently leading to disseminated intravascular coagulation (DIC). Nearly half of the cases involved decompensated liver disease leading to sepsis, often requiring intensive care unit admission with vasopressor support, exacerbating ischaemia. Clinical features included retiform purpura, widespread ecchymosis, blistering, necrosis and, in severe cases, autoamputation of digits. Commonly affected areas were the abdomen, lower limbs and digits, with frequent genital involvement. Histopathology distinguished PF from mimics like toxic epidermal necrolysis and necrotizing fasciitis, showing hallmark microthrombi, as well as red blood cell extravasation and epidermal necrosis, which are consistent with thrombotic vasculopathy and DIC. Haematological abnormalities included thrombocytopenia, prolonged coagulation times, hypofibrinogenemia and elevated liver markers. Management involved multidisciplinary care, addressing the underlying pathology, reversing coagulopathy and providing intensive skin care. Despite these measures, outcomes were poor; 12 of 19 patients succumbed to multiorgan failure, often secondary to septic shock. Survivors required rehabilitation and often faced long-term sequelae, including amputations or scarring. In conclusion, PF remains a life-threatening complication with high morbidity and mortality, predominantly affecting patients with decompensated liver failure and severe sepsis leading to DIC. Early recognition, prompt intervention and aggressive management of underlying causes are essential to improve survival. This series underscores the need for a multidisciplinary approach and heightened clinical suspicion in high-risk populations. Future research should aim to establish standardized treatment protocols to mitigate the severe outcomes associated with PF.

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Publication Details

Journal
British Journal of Dermatology
Published
2025-06-27
DOI
https://doi.org/10.1093/bjd/ljaf085.324
Primary Topic
Venous Thromboembolism Diagnosis and Management
Type
article
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article

DP01 (P038) Clinical and histopathological features of purpura fulminans: a retrospective case series

D. Creamer, Shafiah Muna Abdul Gafoor, Amelia Gelson-Thomas
British Journal of Dermatology
Venous Thromboembolism Diagnosis and Management
article

DP01 (P038) Clinical and histopathological features of purpura fulminans: a retrospective case series

D. Creamer, Shafiah Muna Abdul Gafoor, Amelia Gelson-Thomas
article en

Abstract

Abstract Purpura fulminans (PF) is a rare but serious condition characterized by purpura and skin necrosis resulting from a hypercoagulable state. This study aimed to evaluate the clinical, histopathological and laboratory features, alongside management approaches and outcomes, in a series of patients diagnosed with PF. By analysing multiple cases, the study seeks to elucidate patterns in presentation and disease progression with the aim to improve diagnostic accuracy and clinical outcomes. We conducted a retrospective analysis of 19 patients presenting with PF at a tertiary care centre. Data were gathered to include clinical notes, laboratory results, histopathology reports and photographic documentation of skin lesions. The cohort spanned all age groups, from infants to older adults. Key variables analysed included clinical provenance, skin findings, haematological parameters, histopathological results, comorbidities, management and outcomes. Statistical analysis was descriptive, focusing on patterns and correlations. The cohort ranged in age from 3 months to 74 years, with 11 male and eight female patients. The primary triggers for PF included severe sepsis secondary to bacterial, viral and fungal infections, frequently leading to disseminated intravascular coagulation (DIC). Nearly half of the cases involved decompensated liver disease leading to sepsis, often requiring intensive care unit admission with vasopressor support, exacerbating ischaemia. Clinical features included retiform purpura, widespread ecchymosis, blistering, necrosis and, in severe cases, autoamputation of digits. Commonly affected areas were the abdomen, lower limbs and digits, with frequent genital involvement. Histopathology distinguished PF from mimics like toxic epidermal necrolysis and necrotizing fasciitis, showing hallmark microthrombi, as well as red blood cell extravasation and epidermal necrosis, which are consistent with thrombotic vasculopathy and DIC. Haematological abnormalities included thrombocytopenia, prolonged coagulation times, hypofibrinogenemia and elevated liver markers. Management involved multidisciplinary care, addressing the underlying pathology, reversing coagulopathy and providing intensive skin care. Despite these measures, outcomes were poor; 12 of 19 patients succumbed to multiorgan failure, often secondary to septic shock. Survivors required rehabilitation and often faced long-term sequelae, including amputations or scarring. In conclusion, PF remains a life-threatening complication with high morbidity and mortality, predominantly affecting patients with decompensated liver failure and severe sepsis leading to DIC. Early recognition, prompt intervention and aggressive management of underlying causes are essential to improve survival. This series underscores the need for a multidisciplinary approach and heightened clinical suspicion in high-risk populations. Future research should aim to establish standardized treatment protocols to mitigate the severe outcomes associated with PF.

British Journal of DermatologyVol. 193(Supplement_1)
King's College Hospital (GB)
Openalex Percentile: Top 70%
Venous Thromboembolism Diagnosis and Management
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