Purpura fulminans lesion on left calf of newborn.

The child above is not an actual patient, and is portrayed here for illustration purposes only. The depiction of a purpura fulminans lesion is from an actual SCPCD patient.*

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

Protein C Deficiency:

Is it acquired or congenital?

SCPCD - Key Management Information

SCPCD in brief: the cause, the diagnosis, the treatment

Which tests may help an early diagnosis of Severe Congenital Protein C Deficiency (SCPCD)

How to Test
for SCPCD

There are several tests that may help you determine whether your patient has SCPCD.

Options for managing treatment of Severe Congenital Protein C Deficiency (SCPCD)

Options for
Management
of SCPCD

Options include protein C replacement, anticoagulation and liver transplantation.⁵

References:

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.

* Purpura fulminans photo above courtesy of Bubl B, et al. Neonatal Purpura Fulminans, Swiss Society of Neonatology, University Children's Hospital of Berne, Switzerland.

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