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