Two Taiwanese siblings with dyschromatosis universalis hereditaria.

Two Taiwanese siblings presented with clinical and histological findings of dyschromatosis universalis hereditaria, which is an uncommon hereditary skin disease. A 26-year-old Taiwanese man had developed diffuse hyperpigmentation with hypopigmented spots over his whole body from the age of 4 years. His 34-year-old brother had also developed a similar skin pigmentary defect from about the same age. Histological examination of the hyperpigmented lesions found an increase in melanin in the basal layer, pigmentary incontinence, and some melanophages in the upper dermis but no increase in the number of melanocytes. The unique clinical, histological, and genetic features of this condition are outlined.