2013
DOI: 10.1590/s0365-05962013000200001
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Abstract: Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB … Show more

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Cited by 44 publications
(55 citation statements)
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References 40 publications
(85 reference statements)
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“…Además, cerca del 50-80% de los pacientes con EBDR, desarrollan a lo largo de su vida carcinoma cutáneo de células escamosas (especialmente luego de la segunda década de vida) [3] . El desarrollo de ERC es una de las complicaciones más frecuentes de los pacientes con EB, siendo responsable del 12% de las muertes de los mismos [7] . Los pacientes con EBDR y ERC terminal generalmente son tratados con hemodiálisis o diálisis peritoneal como TRR, siendo poco conocido el rol del trasplante renal en los mismos [5] .…”
Section: Discussionunclassified
“…Además, cerca del 50-80% de los pacientes con EBDR, desarrollan a lo largo de su vida carcinoma cutáneo de células escamosas (especialmente luego de la segunda década de vida) [3] . El desarrollo de ERC es una de las complicaciones más frecuentes de los pacientes con EB, siendo responsable del 12% de las muertes de los mismos [7] . Los pacientes con EBDR y ERC terminal generalmente son tratados con hemodiálisis o diálisis peritoneal como TRR, siendo poco conocido el rol del trasplante renal en los mismos [5] .…”
Section: Discussionunclassified
“…Medical surveillance for involvements of other systems should be a part of the rou ne evalua on. In children, complica ons such as: cutaneous losses, infec ons, anemia, respiratory failure, dehydra on or diges ve problems lead to impaired nutri on and growth and is some cases even death 4,12,16 . Despite the progress on the knowledge about the cause of EB, currently, there is no sa sfactory treatment available and the long-term prognosis of the disease is poor.…”
Section: Discussionmentioning
confidence: 99%
“…Gene, protein replacement and cell-based therapies are being inves gated, but these approaches are at the early stages of inves ga on, and there are a number of uncertain es surrounding them 13,17 . Treatment is primarily suppor ve and preven ve consis ng of: wound healing, infec on control, pain management, nutri onal support, adequate dressing and other therapies depending on the complica ons that arise 4,12,17 .…”
Section: Discussionmentioning
confidence: 99%
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“…However, patients with EB have a substantial risk of developing basal cell carcinomas (Boeira et al, 2013).…”
mentioning
confidence: 99%