title: [Genetics of Hailey-Hailey familial chronic benign pemphigus].
abstract: 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.
authors.affiliation: Klinik und Poliklinik für Hautkrankheiten, Medizinischen Akademie Erfurt.
authors.forename: G
authors.initials: G
authors.lastname: Richard
authors.forename: R
authors.initials: R
authors.lastname: Linse
authors.forename: J
authors.initials: J
authors.lastname: Hadlich
authors.forename: H
authors.initials: H
authors.lastname: Schubert
mesh_terms: D005260:Female; D006580:Genetic Carrier Screening; D005819:Genetic Markers / Q000235:genetics; D005838:Genotype; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010392:Pemphigus / Q000175:diagnosis / Q000235:genetics*; D010641:Phenotype
chemical_list: D005819:Genetic Markers
vernacular_title: Zur Genetik des Pemphigus benignus chronicus familiaris Hailey-Hailey.