[Genetics of Hailey-Hailey familial chronic benign pemphigus]

Starting from 12 index patients with familial benign chronic pemphigus from 11 kindreds, systematic kindred examinations were carried out with 182 relatives. 11 pedigrees were established. In this process 6 patients out of 3 kindreds were identified additionally. 10 genotypic carriers of the disease were found of 30 examined relatives by a newly developed UV-provocation test. An autosomal-dominant hereditary pattern was proven for sure and with high probability, respectively, in 10 out of 11 kindreds by formal genetic genealogy-examinations. There was an incomplete penetrance in 3 kindreds. A sex-determined heredity of disease can be excluded. There was no association between familial benign chronic pemphigus and HLA-A, B, C-system.