Successful Management of Neonatal Purpura Fulminans Caused by Congenital Protein C Deficiency: A Case Report

Neonatal purpura fulminans, often caused by congenital protein C deficiency, presents with rapid onset of skin necrosis and microvascular thrombosis and can progress to disseminated intravascular coagulation. Therefore, early diagnosis and intervention are crucial for patient survival. We report a case of a neonate who developed purpura fulminans within hours after birth and presented with skin lesions on the right lower leg and abnormal coagulation markers. Treatment with fresh frozen plasma and heparin was promptly initiated, leading to significant improvement. Conservative management with ointments and wound dressings was also administered to treat the skin lesions. Genetic testing confirmed a compound heterozygous PROC mutation. Despite severe ocular complications that resulted in blindness, limb function was preserved. This case highlights the importance of early recognition, genetic testing, and aggressive treatment in managing neonatal purpura fulminans caused by congenital protein C deficiency to improve outcomes and prevent severe sequelae.

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Publication Details

Journal
Journal of Plastic and Reconstructive Surgery
Published
2025-08-12
DOI
https://doi.org/10.53045/jprs.2025-0027
Primary Topic
Blood Coagulation and Thrombosis Mechanisms
Type
article
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article

Successful Management of Neonatal Purpura Fulminans Caused by Congenital Protein C Deficiency: A Case Report

Narihito Yoshioka, Makiko Shiraishi
Journal of Plastic and Reconstructive Surgery
Blood Coagulation and Thrombosis Mechanisms
article

Successful Management of Neonatal Purpura Fulminans Caused by Congenital Protein C Deficiency: A Case Report

Narihito Yoshioka, Makiko Shiraishi
article en

Abstract

Neonatal purpura fulminans, often caused by congenital protein C deficiency, presents with rapid onset of skin necrosis and microvascular thrombosis and can progress to disseminated intravascular coagulation. Therefore, early diagnosis and intervention are crucial for patient survival. We report a case of a neonate who developed purpura fulminans within hours after birth and presented with skin lesions on the right lower leg and abnormal coagulation markers. Treatment with fresh frozen plasma and heparin was promptly initiated, leading to significant improvement. Conservative management with ointments and wound dressings was also administered to treat the skin lesions. Genetic testing confirmed a compound heterozygous PROC mutation. Despite severe ocular complications that resulted in blindness, limb function was preserved. This case highlights the importance of early recognition, genetic testing, and aggressive treatment in managing neonatal purpura fulminans caused by congenital protein C deficiency to improve outcomes and prevent severe sequelae.

Journal of Plastic and Reconstructive Surgery
Osaka Women's and Children's Hospital (JP)
Zero hunger
Openalex Percentile: Top 75%
Blood Coagulation and Thrombosis Mechanisms
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Successful Management of Neonatal Purpura Fulminans Caused by Congenital Protein C Deficiency: A Case Report — Narihito Yoshioka, Makiko Shiraishi · Journal of Plastic and Reconstructive Surgery (2025) | TGRS Research Map | TGRS