Pigmented Xerodermoid: first report of a family

Pigmented Xerodermoid is a variant of Xeroderma pigmentosum characterized by late manifestation of sun--sensitivity, dyschromic and poikilodermatic skin changes and multiple malignant skin tumors in light exposed areas. In contrast to Xeroderma pigmentosum excision repair system is normal, but DNA synthesis after UV--irradiation is markedly prolonged, suggesting that the postreplicational repair mechanism is disturbed. A family of 89 members with 4 siblings suffering from pigmented Xerodermoid was examined. The pedigree of this family suggests autosomal recessive inheritance in this disease.