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In a patient with total C2 deficiency and repeated respiratory infections a papulovesicular rash showed to be due to leukocytoclastic vasculitis. The patient's brother also had a total deficiency of C2 but no clinical symptoms. HLA-investigation in both him and the proband showed the genotype commonly reported in cases with homozygous C2 deficiency. Their heterozygous mother and sister had slightly decreased C2 levels.
Irestedt et al. (Fri,) studied this question.