An association between C1 esterase inhibitor deficiency and lupus erythematosus: report of two cases and review of the literature.

Two patients who had lupus erythematosus and C1 esterase inhibitor deficiency are described. The data on eleven previously reported cases are reviewed and summarized with those from our own cases. Early complement component depletion secondary to C1 esterase inhibitor deficiency may predispose to the development of lupuslike syndromes by impairing the ability of the organism to handle foreign antigen. Further study of this unique group of patients may provide insight into the pathogenesis of immune complex disease.