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

Lemesle, F G; Gris, J C; Schwed, J F; Arich, C · Intensive Care Med · 1993

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.

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