We report a Japanese family with dyschromatosis symmetrica hereditaria (DSH) (MIM 127400 in McKusick's Mendelian Inheritance in Man), a rare autosomal dominant genodermatosis, predominantly occurring among Japanese and Korean individuals. Members of the present family affected with the disease showed a mixture of hyperpigmented and hypopigmented macules distributed on the face and the dorsal aspects of the extremities, which are typical of DSH. As most of the literature on DSH has been written in Japanese, dermatologists outside Japan are not familiar with the condition. In this paper, 185 cases of DSH, most of them reported in Japanese, are reviewed and unique clinical, histological and genetic features of this condition are delineated.
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