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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 rapid protein C test can help physicians diagnose and manage this rare condition, reducing morbidity and saving the lives of infants.2,3

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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 survivors.1,3 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

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Scpcd7

SCPCD action tool

How to proceed if you’re facing purpura fulminans and must determine whether it's due to sepsis or SCPCD.

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References:

  1. Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.

  2. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

  3. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  4. 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.

  5. 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.

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