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Review
. 1993;19(2):115-6.
doi: 10.1007/BF01708373.

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

F G Lemesle  1 , J C Gris, J F Schwed, C Arich
Affiliations
Review

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

F G Lemesle et al. Intensive Care Med. 1993.

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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