Kimura's disease and Behcet's syndrome in the same family – are they associated?

BackgroundBehcet's disease (BD) and Kimura's disease (KD) are two inflammatory diseases commonly found in Japan. In Israel, both diseases are quite rare. Recently, we encountered a family whose three siblings suffer from BD and an additional brother suffers from KD. This observation raised the question as to whether both diseases have a common underlying genetic basis.Aim of the studyTo describe this unique family and to search for possible common alleles in the IL10 gene between BD and KD, as several SNPs in this gene are known to be associated with BD.MethodsThree BD siblings and their brother with KD were interviewed and examined. Genomic DNA was prepared from blood samples taken from all nine members of the family. The DNA was genotyped for sequence variations of six SNPs on the IL10 gene.ResultsThe IL10 SNPs did not segregate with BD and KD suggesting that there was no association between the IL10 gene and these diseases in this family.ConclusionsSNPs in the IL10 gene shown to be susceptibility factors in adult BD were not associated with BD in this family. The question regarding a possible common genetic basis for KD and BD requires further investigation.