Severe protein C deficiency in a newborn.
An infant presented at birth with a cutaneous lesion that developed the characteristics of purpura fulminans. He sustained a cerebral infarction during the first 4 days of life, which was initially misinterpreted to be cerebral hemorrhage resulting from thrombocytopenia and hypofibrinogenemia. After the diagnosis of Protein C deficiency was made and effective replacement therapy begun, no further cutaneous lesions occurred. Although there was no evidence of further cerebral infarction, the initial insult progressed to severe encephalomalacia and cerebral atrophy. This disorder should be considered in infants with purpura fulminans or those with cerebral infarction, as prompt institution of transfusion therapy to replace the missing protein may prevent further damage.
Pegelow
C H
CH
Department of Pediatrics, University of Miami School of Medicine, Miami, Florida 33101.
Curless
R
R
Bradford
B
B
D006801:Humans; D007231:Infant, Newborn; D008297:Male; D020151:Protein C Deficiency; D011488:Protein Deficiency / Q000175:diagnosis*; D011693:Purpura / Q000209:etiology