Lack of linkage between hereditary angioedema and the A and B loci of the HLA system.

A large 81 member, four-generation black family with hereditary angioedema is reported with regards to its clinical course and the association with the histocompatibility system. No mortality was seen related directly to the disease. Assuming that this trait is autosomal dominant it appeared to have no linkage with the histocompatibility system as noted by an estimated recombination rate in males and females of 0.5 and a maximum lod score of 0.0. Further evidence of no linkage is given by the fact that lod scores below -3.0 were observed for values of theta less than 0.5.