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Revista Cubana de Pediatría

versión impresa ISSN 0034-7531

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TANO ESPINOSA, Ernesto Melardo. The xeroderma pigmentosum in its phase of tumoral cutaneous proliferation. Rev Cubana Pediatr [online]. 2012, vol.84, n.1, pp.103-116. ISSN 0034-7531.

The xeroderma pigmentosum is a rare dermatologic disease, autosomal recessive and its etiology is related to the congenital deficit of an endonuclease inactivating the DNA of the cutaneous cells muting by the incidence of ultraviolet rays. The disease passes by three clinical, evolutionary and morphological stages. If the patients are not appropriately protected of the sunlight, they suffer an accelerated aging of the skin and inevitably develop ocular and dermal lesions that may to lead to multiple cancerous lesions. Nowadays, there is not a curative treatment. The early diagnosis and the protection the ultraviolet rays are the essential factors in the treatment of the involved patients. Authors describe the clinical evolution of a girl aged 10 presenting with xeroderma pigmentosum with a very advanced phase of the disease and a significant growth of cutaneous carcinomas. The objective of this paper is to present a uncommon clinical case not much frequent of xeroderma pigmentosum and at the same time, to make a bibliographic review to direct towards the early diagnosis and the appropriate treatment in this type of cases.

Palabras clave : xeroderma pigmentosum; photosensitivity; skin cancer.

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