Rapid recovery of acquired purpura fulminans in a patient with familial C4bBP deficiency.
A 32-year-old pregnant woman developed meningococcemia associated purpura fulminans and quickly improved with therapy. After this disease C4b-Binding Protein (C4bBP) plasma levels remained very low while protein S activity was in the normal range. Familial investigation proved a hereditary C4bBP deficiency. This observation points out the role of the protein C-protein S system during acquired purpura fulminans.
Lemesle
F G
FG
Service de Réanimation, Hôpital Hoche, Nimes, France.
Gris
J C
JC
Schwed
J F
JF
Arich
C
C
D000328:Adult; D000990:Antithrombin III / Q000032:analysis; D016470:Bacteremia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002352:Carrier Proteins / Q000097:blood*; D015935:Complement C4b / Q000032:analysis*; D003169:Complement Inactivator Proteins; D005260:Female; D006023:Glycoproteins; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000097:blood* / Q000151:congenital / Q000235:genetics; D008589:Meningococcal Infections / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011247:Pregnancy; D011251:Pregnancy Complications, Infectious / Q000175:diagnosis / Q000188:drug therapy; D011486:Protein C / Q000032:analysis; D017293:Protein S / Q000097:blood; D018455:Protein S Deficiency; D011693:Purpura / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D011486:Protein C; D017293:Protein S; D015935:Complement C4b; D000990:Antithrombin III