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  • Title: Pigmented Xerodermoid: first report of a family.
    Author: Hofmann H, Jung EG, Schnyder UW.
    Journal: Bull Cancer; 1978; 65(3):347-50. PubMed ID: 719186.
    Abstract:
    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.
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