Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasília, Campus Universitário Darcy Ribeiro, Brasília, Federal District, 70910-900, Brazil.
Clinical Nutrition Unit, University Hospital of Brasília, Brasília, Federal District, Brazil.
Qual Life Res. 2020 Jul;29(7):1731-1745. doi: 10.1007/s11136-020-02495-5. Epub 2020 Apr 3.
Individuals with epidermolysis bullosa (EB) present with various clinical manifestations of different severities that affect quality of life (QoL). This systematic review synthesizes the current evidence about the QoL of individuals with EB.
We included observational studies with people of all age groups, both sexes, and any EB type. Studies with qualitative methodology, chapters of books, meeting proceedings, and abstracts were excluded.
In this study, 12 articles comprising 745 individuals were included. More than half of the articles observed lower QoL in individuals with recessive dystrophic EB (RDEB) or junctional EB (JEB). Three articles indicated that EB affected QoL more in women than in men, and one article identified that children with EB suffered more than adults with the disease. Pain was frequently reported. Seven articles identified difficulty in sports, two identified a need for bathing assistance, and three identified eating difficulties. Additionally, participants reported that family relationships and friendships were affected, and they experienced feelings of anxiety and depression. Some of the instruments used evaluated QoL in general dermatologic conditions, and one was specific to EB.
QoL is more affected in people who have RDEB and JEB. Regarding sex and age, women and children need special care in their monitoring. It is necessary that guidelines on pain management be more disseminated and put into practice. Future studies should use standardized specific instruments to assess the QoL in EB individuals, while considering the particularities of the different age groups.
患有大疱性表皮松解症（EB）的个体表现出不同严重程度的各种临床表现，这些临床表现会影响生活质量（QoL）。本系统综述综合了目前关于 EB 个体生活质量的证据。
我们纳入了观察性研究，研究对象为所有年龄段、男女两性和任何类型的 EB 患者。排除了定性方法学研究、书籍章节、会议记录和摘要。
在这项研究中，有 12 篇文章纳入了 745 名患者。超过一半的文章观察到隐性营养不良性大疱性表皮松解症（RDEB）或交界性大疱性表皮松解症（JEB）患者的生活质量较低。有 3 篇文章表明，EB 对女性的生活质量影响大于男性，有 1 篇文章表明，EB 患儿比成年患者遭受的痛苦更大。经常报告疼痛。有 7 篇文章确定运动存在困难，2 篇文章确定需要沐浴协助，3 篇文章确定存在饮食困难。此外，参与者报告家庭关系和友谊受到影响，他们还感到焦虑和抑郁。一些使用的工具评估了一般皮肤病情况下的生活质量，还有一个工具是专门针对 EB 的。
RDEB 和 JEB 患者的生活质量受影响更大。关于性别和年龄，女性和儿童在监测时需要特别关注。有必要更广泛地传播和实施关于疼痛管理的指南。未来的研究应该使用标准化的特定工具来评估 EB 个体的生活质量，同时考虑到不同年龄组的特殊性。
