Purpura fulminans in a Chinese boy with congenital protein C deficiency.
Severe and recurrent purpura fulminans developed in a Chinese boy at one day of age. Results of coagulation studies performed on the patient during attacks were compatible with the diagnosis of disseminated intravascular coagulation. Subsequent investigations have revealed that the patient is homozygous and that his parents are heterozygous for protein C deficiency. Cryoprecipitate and fresh frozen plasma induced a remission, and administration of warfarin has been successful in preventing recurrence of attacks for as long as 8 months without infusion of any plasma components. None of the family members who are heterozygous for protein C deficiency have had thrombotic episodes.
D003131:Combined Modality Therapy; D003450:Cryoglobulins / Q000627:therapeutic use; D006023:Glycoproteins / Q000172:deficiency*; D006493:Heparin / Q000627:therapeutic use; D006720:Homozygote; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009336:Necrosis / Q000473:pathology; D010949:Plasma; D011486:Protein C; D011488:Protein Deficiency / Q000150:complications / Q000235:genetics*; D011693:Purpura / Q000150:complications* / Q000473:pathology / Q000628:therapy; D012867:Skin / Q000473:pathology; D014859:Warfarin / Q000627:therapeutic use
D003450:Cryoglobulins; D006023:Glycoproteins; D011486:Protein C; D014859:Warfarin; D006493:Heparin