Abstract
Several cases of isolated C2 [the 2nd complement component] deficiency in man have been reported. The earliest cases did not seem to be associated with known diseases or syndromes; more recently, reports of C2 deficiency associated with systemic lupus erythematosus, anaphylactoid purpura, recurrent infections, and dermatomyositis have appeared. The propositus, a 24 yr old woman with a lupus erythematosus-like rash, a history of arthralgla and a selective deficiency of C2. Studies of hemolytic C2 of the immediate members of her family indicate an autosomal-recessive mode of inheritance. A C2 deficiency apparently predisposes some persons to serious vascular diseases.