Resumen de Epidermólisis ampollosa. Artículo monográfico

Belén Anso de Miguel, Marina Georgieva Raycheva, Sonia Gracia Orea, Belén González Abengochea, Ana Cid Samper, Irama Iguaz Marco

• español

  Se buscaron artículos en las bases de datos “Pubmed” y “Embase”, y en el metabuscador “Epistemonikos”.

  En Pubmed se buscó la palabra clave: “Epidermolysis Bullosa,” y se aplicaron los filtros: artículos de menos de 5 años de antigüedad, metaanálisis y revisiones sistemáticas. De los 27 resultados se seleccionaron tres 1,3,4.

  En Embase, buscando la misma palabra clave, y con los filtros: artículos de menos de 5 años de antigüedad, metaanálisis y revisiones sistemáticas. De los 47 resultados se seleccionó 1 revisión sistemática 7.

  En Epistemonikos se buscó “Epidermolysis Bullosa”, filtrando para obtener revisiones sistemáticas realizadas en los últimos 10 años. De los 51 resultados se seleccionaron 4 5,6,8,9.

  La epidermólisis ampollosa, también conocida como “Piel de mariposa”, es una enfermedad poco frecuente caracterizada por fragilidad de la piel y de las membranas mucosas. Cualquier roce mecánico mínimamente traumático origina la formación de ampollas. Es de etiología genética y a día de hoy no se conoce cura.

  Esta enfermedad, de varios subtipos que varían en gravedad y extensión, cursa generalmente con lesiones, infecciones y carcinomas de la piel, dolor, prurito y cicatrices que alteran la movilidad general. Unido a las alteraciones nutricionales asociadas a las lesiones y problemas gastrointestinales, producen un deterioro de la calidad de vida de los niños y adolescentes afectados.

  Aspectos importantes a considerar son el aporte nutricional, el cuidado proporcionado por y a familiares, la implicación de los profesionales sanitarios y la formación de personal de enfermería que pueda abordar esta enfermedad sistémica de la mejor forma posible.

• English

  Articles were searched in the «Pubmed» and «Embase» databases, and in the «Epistemonikos» metasearch engine.

  The keyword «Epidermolysis Bullosa» was searched in Pubmed, and filters were applied: articles less than 5 years old, meta-analyses, and systematic reviews. Of the 27 results, three 1,3,4 were selected.

  In Embase, searching for the same keyword, and with the filters: articles less than 5 years old, meta-analyses and systematic reviews. Of the 47 results, 1 systematic review 7 was selected.

  In Epistemonikos, “Epidermolysis Bullosa” was searched, filtering to obtain systematic reviews carried out in the last 10 years. Of the 51 results, 4 5,6,8,9 were selected.

  Epidermolysis bullosa, also known as «Butterfly skin», is a rare disease characterized by fragility of the skin and mucous membranes. Any minimally traumatic mechanical friction causes the formation of bottles. It is of genetic etiology and today there is no known care.

  This disease, of several subtypes that vary in severity and extent, generally presents with lesions, infections and skin carcinomas, pain, itching and scars that alter general mobility. Together with the nutritional alterations associated with the injuries and gastrointestinal problems, they produce a deterioration in the quality of life of the affected children and adolescents.

  Important aspects to consider are the nutritional contribution, the care provided by family members, the involvement of health professionals and the training of nursing personnel who can address this systemic disease in the best possible way.

  Epidermolysis bullosa, also known as «butterfly skin», is a rare disease characterized by fragility of the skin and mucous membranes. Any minimally traumatic mechanical friction causes the formation of blisters. It is of genetic etiology and to date there is no known cure.

  This disease, of various subtypes varying in severity and extent, generally presents with skin lesions, infections and carcinomas, pain, pruritus and scars that alter general mobility. Together with the nutritional alterations associated with the lesions and gastrointestinal problems, they produce a deterioration in the quality of life of affected children and adolescents.

  Important aspects to consider are nutritional support, the care provided by and to family members, the involvement of healthcare professionals and the training of nursing staff who can deal with this systemic disease in the best possible way.