General treatment and wound management in hereditary epidermolysis bullosa: indication and experience using silver hydrofiber dressing / Tratamento geral e das feridas na epidermólise bolhosa hereditária: indicação e experiência usando curativo de hidrofibra com prata

Introduction: Hereditary epidermolysis bullosa (EB) is a rare disorder characterized by cutaneomucous fragility, with formation of blisters during minimal trauma. Treatment consists of clinical and nutritional support and management of pain and skin lesions. Silver hydrofiber (Aquacel Ag®) is a type of carboxymethylcellulose fiber dressing with silver that can be used in selected cases of EB. Objective: To review the literature on the general treatment and management of cutaneous lesions in congenital EB and evaluate the indication and experience of using silver hydrofiber dressing. Methods: The review included original articles and systematic reviews published between 2009 and 2014. We also selected two patients with congenital EB treated at the Plastic Surgery Division of Hospital das Clínicas of the Faculty of Medicine of Ribeirão Preto at the University of São Paulo. Results: There is a shortage of scientific evidence related to the treatment of skin lesions in congenital EB, with most recommendations being based on expert opinions. Hydrofiber is indicated in most consensuses for wounds with some exudation and has been shown to be more absorbent than alginate. In our experience, there was apparent improved control of pain, bleeding, and hypothermia with the use of hydrofiber, which has the advantage of not requiring daily changes and can remain on the wound for up to two weeks. Conclusions: The general and lesion treatments in EB are challenging. Hydrofiber with silver is a treatment option for wounds in hereditary EB, without the need for daily dressing changes.

Introdução: Epidermólise bolhosa (EB) hereditária é uma desordem rara caracterizada pela fragilidade cutaneomucosa, com formação de bolhas ao mínimo trauma. O tratamento consiste em suporte clínico, nutricional, manejo da dor e das lesões cutâneas. A hidrofibra com prata (Aquacel Ag®) é um tipo de curativo de fibra de carboximetilcelulose e prata que pode ser utilizada em casos selecionados de EB. Objetivo: Revisão da literatura sobre o tratamento geral e o manejo das lesões cutâneas na EB congênita, além de avaliar a indicação e experiência usando curativo de hidrofibra com prata. Métodos: A revisão incluiu artigos originais e revisões sistemáticas, publicados entre 2009 e 2014. Selecionamos ainda dois pacientes com EB congênita tratados na Divisão de Cirurgia Plástica do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo. Resultados: Há escassez de evidências científicas relacionadas ao tratamento das lesões cutâneas na EB congênita, sendo a maioria das recomendações baseadas em opiniões de especialistas. A hidrofibra está indicada na maioria dos consensos para feridas com alguma exsudação e mostrou-se mais absorvente que o alginato. Em nossa experiência, houve aparente melhor controle da dor, do sangramento e da hipotermia com o uso da hidrofibra, que apresenta a vantagem de não necessitar de trocas diárias, podendo permanecer na ferida por até duas semanas. Conclusões: O tratamento geral e das lesões na EB é um desafio. A hidrofibra com prata é uma opção de tratamento para as feridas na EB hereditária, sem necessidade de trocas diárias de curativo.

Nutritional Outcomes in Children with Epidermolysis Bullosa: The Experiences of Two Centers in Korea

PURPOSE: Epidermolysis bullosa (EB) is associated with variable risks of extracutaneous manifestations and death. Currently, there is limited information on the clinical course and prognosis of EB in Korea. This study analyzed the nutritional outcomes, clinical morbidity, and mortality of children with EB. MATERIALS AND METHODS: Thirty patients, admitted to Severance Hospital and Gangnam Severance Hospital, from January 2001 to December 2011, were retrospectively enrolled. All patients were diagnosed with EB classified by dermatologists. RESULTS: Among the 30 patients, 5 patients were diagnosed with EB simplex, four with junctional EB, and 21 with dystrophic EB. Wound infection occurred in 47% of the patients, and blood culture-proven sepsis was noted in 10% of the patients. Two (9.2%) patients had esophageal stricture and 11 (52.4%) of the dystrophic EB patients received reconstructive surgery due to distal extremity contracture. There were five mortalities caused by sepsis, failure to thrive, and severe metabolic acidosis with dehydration. According to nutrition and growth status, most of the infants (97%) were born as appropriate for gestational age. However, at last follow-up, 56% of the children were below the 3rd percentile in weight, and 50% were below the 3rd percentile in weight for height. Sixty percent of the children had a thrive index below -3. CONCLUSION: Postnatal growth failure is a serious problem in children with EB. Strategies to maximize nutritional support could alleviate growth failure in children with EB, and thus improve clinical outcomes.

Epidermólise bolhosa: revisäo da literatura e descriçäo de três casos / Epidermolysis bullosa: report of three cases and review of the literature

Neste trabalho säo descritos três casos de pacientes encaminhados ao Serviço de Radiologia do Hospital das Clínicas da UFMG com diagnóstico de epidermólise bolhosa e disfagia. É feita revisäo da literatura, com ênfase para as manifestaçöes gastrointestinais e alteraçöes radiológicas da doença