FAMILIAL PROPERDIN DEFICIENCY ASSOCIATED WITH CHRONIC DISCOID LUPUS-ERYTHEMATOSUS
- 1 April 1989
- journal article
- research article
- Vol. 76 (1) , 76-81
Abstract
A large family comprised of 18 members is described. Four male members are properdin-deficient, all are healthy bar the index patient who presented with chronic discoid lupus erythematosus. Serum from properdin-deficient males had a reduced ability to lyse rabbit erythrocytes via the alternative pathway or solubilize pre-formed immune complexes. Addition of purified properdin restored these activities. Classical pathway activity was normal. Definite, probable and possible female carriers had normal classical and alternative pathway activities.This publication has 21 references indexed in Scilit:
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