Abstract
Resumo Relato de um caso clinico de Xeroderma Pigmentoso com carcinoma espinocelular de conjuntiva bilateral que apresentou regressao importante das dimensoes tumorais com o uso de Interferon alfa-2b topico. Relato de caso: Paciente feminina com Xeroderma Pigmentoso em estagio avancado, com ausencia de pele sadia, tendo sido submetida a cerca de 60 exereses de lesoes de pele malignas. A paciente compareceu com tumoracao conjuntival em ambos os olhos, correspondendo a carcinoma espinocelular de conjuntiva e neoplasia intraepitelial de conjuntiva em olho esquerdo. Devido as dificuldades cirurgicas, alta taxa de recidiva e elevada probabilidade de formacao de simblefaro foi-se iniciado terapeutica com Interferon alfa-2beta 1.000.000 unidades topico, obtendo-se bons resultados com importante regressao do tamanho da lesao e resolucao dos sintomas. Conclusao: O uso topico de interferon alfa-2beta em neoplasia escamosa de conjuntiva, mostrou-se uma boa opcao terapeutica em situacoes de elevado risco cirurgico e de complicacoes pos operatorias.
Highlights
Xeroderma Pigmentosum (XP) is an recessive autosomal disorder of DNA repair, of genetically heterogeneous etiology and variable expressivity, resulting in sharp sensitivity to ultraviolet radiation (UV)
Due to its various actions the medication has been used in several areas of medicine
23 of the patients who had undergone some previous treatment for tumor lesion presented recurrence and underwent a new therapy with Interferon alfa-2b
Summary
Xeroderma Pigmentosum (XP) is an recessive autosomal disorder of DNA repair, of genetically heterogeneous etiology and variable expressivity, resulting in sharp sensitivity to ultraviolet radiation (UV). There are eight altered genes described, with variable expressivity of the physical and clinical manifestations being the result of the interaction between genetic risk, genetic heterogeneity and environmental exposure.[1]. It can occur in all ethnic groups with prevalence of 1:1,000,000 in Europe and the US, and 1:22,000 in Japan. The clinical manifestations of XP include extreme sun sensitivity (blistering burns with only a few minutes of exposure) and pigmentation changes (hyperpigmentation and depigmentation), ocular changes, presenting a risk greater than 10,000 times of developing neoplasms in areas exposed to the sun like the skin, mucous membranes and eyes. The patient reported visual and symptom improvement, with VA RE 20/100 and LE 20/400
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