Successful Management of Neonatal Purpura Fulminans Caused by Congenital Protein C Deficiency: A Case Report
Year: 2025
Type: article
Study design: Case Report
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.
Authors: Makiko Shiraishi, Naoto Yoshioka
Institution: Osaka Women's and Children's Hospital
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4413185099