Refined localization of the gene for Clouston syndrome (hidrotic ectodermal dysplasia) in a large French family

Hidrotic ectodermal dysplasia (HED) or Clouston syndrome is a rare autosoma l dominant disorder characterized by nail dystrophy, alopecia and palmoplan tar hyperkeratosis, which maps to chromosome 13q11-q12.1, We confirmed link age of HED to this region in a large French family To define the critical r egion for HED, detailed haplotypes were constructed with new pericentromeri c polymorphic markers. A recombination event in the family indicates that t he HED locus maps centromeric to D13S1832. Our French family does not share a common haplotype with other pedigrees previously published (particularly French-Canadian), indicating that the mutations in these families are like ly to be of different origin.