What is SCPCD?
Severe Congenital Protein C Deficiency is a rare autosomal recessive disorder that leads to high initial mortality and long-term morbidity in survivors.1,2
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
Sometimes, purpura fulminans can hide SCPCD.
A frequent cause of purpura fulminans in neonates is severe infections and associated sepsis. However, Severe Congenital Protein C Deficiency (SCPCD) can also lead to purpura fulminans, with lesions appearing as early as 2-12 hours after birth.1,3 A timely protein C test can help physicians diagnose and manage this rare condition, allowing rapid management that may reduce morbidity and save the lives of infants.2,3
How to Test
There are several tests that may help you determine whether your patient has SCPCD.
Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.
Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.
Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.
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.