Rapid recovery of acquired purpura fulminans in a patient with familial C4bBP deficiency.
case_report · Level V
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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.
Medical subject headings
- Bacteremia
- Carrier Proteins
- Complement C4b
- Complement Inactivator Proteins
- Glycoproteins
- Immunologic Deficiency Syndromes
- Meningococcal Infections
- Pregnancy Complications, Infectious
- Purpura