What is SCPCD?

Severe Congenital Protein C Deficiency is an autosomal, rare condition that leads to high initial mortality and long-term morbidity in survivors.¹
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.

Even if the most frequent cause of purpura fulminans in neonates is severe acute infections and associated 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

Differential Diagnosis

Purpura fulminans:
Is it acquired or congenital protein C deficiency?

Purpura Fulminans - Investigation

Key learnings for the management of purpura fulminans: is it congenital or acquired protein C deficiency?

How to test
for SCPCD?

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

Options for
management
of SCPCD

Treatments include protein C replacement, anti-coagulation 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.
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.

C-ANPROM /INT/ /5460- November 2019

What is SCPCD?

Sometimes, purpura fulminans can hide SCPCD.

Differential Diagnosis

Purpura Fulminans - Investigation

How to test for SCPCD?

Options for management of SCPCD

Interested in severe congenital protein c Deficiency (SCPCD) and its management ? Sign up below !

Sometimes, purpura fulminans
can hide SCPCD.

How to test
for SCPCD?

Options for
management
of SCPCD

Interested in severe congenital protein c
Deficiency (SCPCD) and its management ?
Sign up below !