Differences between purpura fulminans due to Severe Congenital Protein C Deficiency or due to acquired protein C deficiency

Protein C Deficiency: Is It Acquired or Congenital ?

Determining the root cause of purpura fulminans

Ruling out ACQUIRED Protein C (PC) DEFICIENCY as a cause of purpura fulminans (PF)

Diagnosing Severe Congenital Protein C Deficiency (SCPCD) as a root cause of neonatal PF can be difficult. This is a result of the correlation between Disseminated Intravascular Coagulation (DIC) and severe acute infections, sepsis, and SCPCD.¹

For proper diagnosis of SCPCD, determining the underlying cause of PF is crucial.^1,2

acquired VS. congenital protein C (PC) deficiency: Differences in clinical and laboratory findings

PF due to
ACQUIRED PC DEFICIENCY

SKIN LESIONS THAT:¹

Develop in distal extremities and progress proximally
Appear as a generalized or diffuse rash affecting entire body surface

UNDETECTABLE PROTEIN C LEVELS^1,**

ISOLATION OF CAUSATIVE AGENT¹

e.g., Neisseria meningitides, streptococcus pneumoniae, Group A and B streptococci, etc.¹

LABORATORY EVIDENCE OF A SEVERE ACUTE PHASE REACTION¹

RESPONSE TO ANTIMICROBIAL THERAPY¹

Learn more about
the causes of protein C deficiency

PF due to
SCPCD

SKINS LESIONS THAT:¹

Develop on the lower limbs and male genitalia*
Form at pressure points, such as the heels and buttocks

UNDETECTABLE PROTEIN C LEVELS^2,3,**

In carriers of a protein C mutation, protein S levels are higher than non-carriers⁴

CEREBRAL VENOUS THROMBOSIS¹

Blindness arising from vitreal bleeding, retinal vein, artery or vitreal vein thrombosis with retinal detachment in the form of leukocoria or ischemic optic atrophy¹

FREQUENTLY DUE TO CONSANGUINEOUS PARENTS and a POTENTIAL HISTORY of miscarriages¹

Learn more about
the signs and symptoms of SCPCD

*This may also occur in an identical way to postinfectious PF.¹
**However, DIC, which can also indirectly result from sepsis, is associated with reduced Protein C and S levels because of consumption.¹

Differential diagnosis can be PERFORMED by:^#

PC activity ASSAY^1,3,##

Protein S assay^1,## (low PC levels but normal Protein S levels are suggestive of SCPCD)⁴

PC antigen⁵

Full blood count (Hemoglobin, White blood cells, Platelets)^5,6

Fibrinogen^2,5

D-dimer⁵

Prothrombin time (PT)^2,5

Activated partial prothrombin time^2,5

Genetic analysis²

To rule out acquired PC deficiency:

Blood culture, procalcitonin levels⁸ (to test for bacterial infections including Meningococcal, Streptococcus, Haemophilus and Staphylococcus sepsis) and liver function tests^1,5,7,8,###

PC level testing in parents¹

PC activity assay and PC antigen

MRI (to check for cerebral venous thrombosis)¹

Learn more about
how to test for SCPCD

#All the tests mentioned above may not necessarily be done independently but in conjunction to help provide a complete diagnostic picture.
##Interpretation of PC and PS levels may be hindered if emergency treatment like fresh frozen plasma was already given.
###This list does not include all tests to test for the different causes of acquired PC deficiency.

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.
Libourel EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. British Journal of Haematology. 2002;118:615–618.
Khor B, et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440–442.
Tairaku S, et al. Prenatal genetic testing for falmilial severe congenital protein C deficiency. Hum Genome Var. 2015;2.15017
Rhodes A, et al. Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016.
Intensive Care Med. 2017;43(3):304-377.
Wacker C, et al. Procalcitonin as a diagnostic marker for sepsis: a systematic review and meta-analysis.
Lancet Infect Dis. 2013;13(5):426-435.

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Protein C Deficiency: Is It Acquired or Congenital ?

Ruling out ACQUIRED Protein C (PC) DEFICIENCY as a cause of purpura fulminans (PF)

acquired VS. congenital protein C (PC) deficiency: Differences in clinical and laboratory findings

SKIN LESIONS THAT:1

UNDETECTABLE PROTEIN C LEVELS1,**

ISOLATION OF CAUSATIVE AGENT 1

LABORATORY EVIDENCE OF A SEVERE ACUTE PHASE REACTION1

RESPONSE TO ANTIMICROBIAL THERAPY1

SKINS LESIONS THAT:1

UNDETECTABLE PROTEIN C LEVELS2,3,**

CEREBRAL VENOUS THROMBOSIS1

FREQUENTLY DUE TO CONSANGUINEOUS PARENTS and a POTENTIAL HISTORY of miscarriages1

Differential diagnosis can be PERFORMED by:#

PC activity ASSAY1,3,##

Interested in Severe Congenital Protein CDeficiency (SCPCD) and its management?Sign up below!