Rapid recovery of acquired purpura fulminans in a patient with familial C4bBP deficiency

Intensive Care Med. 1993;19(2):115-6. doi: 10.1007/BF01708373.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antithrombin III / analysis
  • Bacteremia / complications*
  • Bacteremia / diagnosis
  • Bacteremia / drug therapy
  • Carrier Proteins / blood*
  • Complement C4b / analysis*
  • Complement Inactivator Proteins*
  • Female
  • Glycoproteins*
  • Humans
  • Immunologic Deficiency Syndromes / blood*
  • Immunologic Deficiency Syndromes / congenital
  • Immunologic Deficiency Syndromes / genetics
  • Meningococcal Infections / complications*
  • Meningococcal Infections / diagnosis
  • Meningococcal Infections / drug therapy
  • Pregnancy
  • Pregnancy Complications, Infectious* / diagnosis
  • Pregnancy Complications, Infectious* / drug therapy
  • Protein C / analysis
  • Protein S / blood
  • Protein S Deficiency
  • Purpura / complications*
  • Purpura / diagnosis
  • Purpura / drug therapy

Substances

  • Carrier Proteins
  • Complement Inactivator Proteins
  • Glycoproteins
  • Protein C
  • Protein S
  • Complement C4b
  • Antithrombin III