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
There are several recommended tests that can 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.
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.