Sometimes, purpura fulminans
can hide SCPCD.
Even if the most frequent cause of purpura fulminans in neonates is sepsis, Severe Congenital Protein C Deficiency (SCPCD) can also lead to this disorder, with lesions appearing as early as 2-12 hours after birth.1,2 A timely protein C test can help physicians diagnose and manage this rare condition, allowing rapid management that can reduce morbidity and save the lives of infants.2,3
What is SCPCD?
Severe Congenital Protein C Deficiency is an autosomal recessive, rare disorder that leads to high initial mortality and long-term morbidity in survivors1,3. In neonates, SCPCD can manifest, as early as 2-12 hours after birth, as purpura fulminans with necrosis of the skin, disseminated intravascular coagulation, arterial and venous thrombosis.2-4
Is it sepsis or SCPCD?
SCPCD action tool
Suggested actions if you’re facing purpura fulminans and must determine whether it's due to sepsis or SCPCD.
There are several recommended tests that can help you determine whether your patient has SCPCD.
Options for management
Treating acute manifestations of SCPCD include protein C replacement, anti-coagulation and liver transplantation.5
Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.
Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.
Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.
Marlar RA, et al. Report on the diagnosis and treatment of homozygous protein C deficiency. Report of the Working Party on Homozygous Protein C Deficiency of the ICTH-Subcommittee on Protein C and Protein S. Thromb Haemost. 1989;61(3):529-31.
Kroiss S, Albisetti M. Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency. Biologics: Targets & Therapy. 2010;4:51–60.