As an autosomal dominant (OMIM 127500) or recessive disorder (OMIM 612715), the genetic loci of DUH have been mapped to chromosome 6q24.2-q25.2 [3] and 12q21-q23 [4] by the linkage analysis, but the molecular basis of DUH had remained unknown until recently when Zhang’s group reported ABCB6 as a causative gene of DUH [5]. Here, ABCB6 is linked to dyschromatosis universalis hereditaria.