Abstract
Background: Xeroderma pigmentosum (XP) is a usually autosomal recessive disorder linked to a deficiency of the enzyme systems of DNA repair. The pathological sensitivity to the sun exposes the patient to develop multiple cancers. Materials and Methods: We conducted a retrospective study over a period from January 2006 to December 2014. A total of twelve patients were enrolled. Results: These were nine male cases and three female, with a sex ratio of 3/1. The average age was 7.9 years, ranging from 1 to 40 years. Consanguinity between the parents was found in ten cases (83.3%). The first non-tumor cutaneous manifestations appeared in eight patients before the age of six months. During follow-up, seven patients, including three (43%) at the age of eight years, died from metastasis. Conclusion: XP is complicated in the development of cancers, even in children, and is linked to the intensity of solar radiation in Niger. Key words: xeroderma pigmentosum; skin cancers; tongue amputation; Niamey; Niger
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