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Dyschromatosis universalis hereditaria is a rare pigmentary disorder initially described in the Japanese literature. The pattern of inheritance is believed to be autosomal dominant, but many sporadic cases have been reported.
We encountered a family in which dyschromatosis universalis hereditaria occurred in seventeen members of three generations. In the two members whom we observed, typical skin lesions were distributed all over the body except palrns and soles. By pedigree analysis, we found an autosomal dominant pattern of inheritance.
The differential diagnosis of the other reticulate pigmentary disorders is discussed with a review of dyschromatosis reported in the Korean literature.