Protein C is a natural anticoagulant. Neonates with severe deficiency of protein C may present with purpura fulminans.


Clinical features of purpura fulminans:

(1) abrupt onset of ecchymoses

(2) widespread thrombosis in blood vessels

(3) fever

(4) skin and organ necrosis

(5) hypotension

(6) thrombocytopenia with laboratory findings of DIC


Features of the protein C deficiency:

(1) The patient will have a marked deficiency of protein C (< 1% of normal), usually associated with a homozygous or double heterozygous deficiency.

(2) The parents are heterozygous for the protein C defect.

(3) Consanguinity may be present in the family.


The patient may have recurrent episodes of purpura fulminans and/or deep vein thrombosis unless anticoagulation therapy is given.


Differential diagnosis:

(1) DIC due to other cause


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