[Xeroderma pigmentosum in negroid children. 5 cases in Mahori children]

Xeroderma pigmentosum (XP) is a rare genodermatosis characterized by a defect in the repair of DNA damage induced by ultra-violet rays. In black people, XP are much less frequent, only 29 cases have been reported (22 in Africa). Through the study of 5 individual cases of xeroderma pigmentosum in children at Mayotte (Indian Ocean), the authors analyze, a XP literature in order to highlight how this genetic disease is surprisingly frequent in the bantou population. Ocular lesions are precursory symptoms, before skin damage. Neoplasia and death occur frequently in the first decade. Leukoplasia and carcinoma of the tip of the tongue are frequent.