Introdução: A epidermólise bolhosa (EB) é uma doença congênita, rara, autossômica dominante, autoimune da pele que pode ter acometimento multissistêmico e levar a malignidade. Ocorre devido a mutações nas proteínas de ancoragem entre a epiderme e a derme, e se caracteriza pela formação de vesículas ou bolhas principalmente em locais de maior atrito. Essa patologia é subdivida em quatro tipos de acordo com a sua proteína mutante: simples, juncional, distrófica e síndrome de Kindler. Além disso, a EB apresenta elevada morbimortalidade pois não há tratamento específico. Objetivos: Realizar um levantamento bibliográfico para descrever e comparar as principais atualizações terapêuticas de caráter imunológico que auxiliam no tratamento da epidermólise bolhosa a fim de conscientizar os profissionais da saúde acerca do tema. Material e métodos: Trata-se de um estudo de revisão de literatura de cunho descritivo e analítico. Foi realizada uma busca eletrônica nas bases de dados Pubmed, Biblioteca Virtual em Saúde, Schoolar Google e Scielo publicados entre 2013 e 2021. As palavras de busca foram: epidermólise bolhosa, tratamento imunológico, epidermolysis bullosa, immunological treatment. Foram selecionados 14 estudos contendo dados sobre atualizações em tratamentos imunológicos para EB. Resultados: Houve predomínio de 8 opções terapêuticas imunológicas: transplante de células tronco alogênicas, infusão de células tronco mesenquimais, transplante de medula óssea, tratamento subcutâneo com fator estimulador de colônia de granulócitos, rituximab, imunoglobulina intravenosa, apremilast e calcipotrol tópico. Desses, a maioria indicou evidências de melhora clínica na redução de 75% na formação de bolhas, remissão prolongada de bolhas e vesículas por mais de 12 meses e boa resposta ao tratamento, elevando assim a qualidade vida dos portadores de EB. No entanto, ainda não existem evidências de cura e apesar da terapêutica atual resultar em queda da mortalidade, essa ainda representa cerca de 15%. Conclusão: A epidermólise bolhosa apresenta baixa prevalência e tem ampla manifestação clínica. O tratamento ideal se relaciona diretamente com a sintomatologia do paciente, subtipo e extensão da patologia. Portanto, tornam-se necessárias abordagens individualizadas para que a terapêutica possa ser viável, eficaz e com reações adversas toleráveis a fim de melhorar a qualidade de vida dos portadores da doença.
Publicado
2021-04-24
Como Citar
Amaru, V. N., & Chavez, F. M. G. (2021). ABORDAGENS TERAPÊUTICAS IMUNOLÓGICAS NO MANEJO DA EPIDERMÓLISE BOLHOSA: UMA REVISÃO DE LITERATURA. Revista Multidisciplinar Em Saúde, 2(2), 31. https://doi.org/10.51161/rems/972
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