XERODERMA PIGMENTOSO: UMA PATOLOGIA RARA

Abstract

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disease transmitted, that  can affect both sexes and any race. In Brazil, it has an incidence of 1 case for every 1 million individuals. Populations with geographic isolation and/or high levels of consanguinity may have a higher incidence, as is the case in the district of Araras, in the city of Faina (GO), where the incidence of XP is 1 case for every 410 inhabitants. Clinically, it is characterized by erythema with scaling and diffuse hyperpigmentation mainly in photo-exposed areas, which generally begins in the first years of life. Such lesions present a high risk of progression to neoplasms, such as basal cell carcinoma, squamous cell carcinoma and melanoma. Prevention is based on the use of sunscreen and protective clothing.