Purpose

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.

Methods

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.

Results

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.

Conclusion

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.

中文翻译：

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大疱表皮松解症患者的生活质量：系统评价。

目的

患有大疱性表皮松解症（EB）的个体表现出影响生活质量（QoL）的不同严重程度的各种临床表现。这项系统的综述综合了有关EB患者QoL的当前证据。

方法

我们纳入了对所有年龄段的人群（包括性别和任何EB类型）进行的观察性研究。定性方法学研究，书籍章节，会议记录和摘要均排除在外。

结果

在这项研究中，包括745个人的12篇文章。超过一半的文章观察到隐性营养不良性EB（RDEB）或交界性EB（JEB）个体的QoL降低。三篇文章指出，EB对女性生活质量的影响大于男性，而一篇文章指出，患有EB的儿童患此疾病的人数多于成年人。疼痛经常被报道。七篇文章指出运动困难，两篇指出需要洗澡帮助，三篇指出饮食困难。此外，参与者报告说，家庭关系和友谊受到影响，并且他们感到焦虑和沮丧。一些用于评估一般皮肤病状况的QoL的仪器，其中一种是针对EB的。

结论

患有RDEB和JEB的人的生活质量受到的影响更大。关于性别和年龄，妇女和儿童在监测中需要特别注意。有必要进一步传播和实施有关疼痛管理的指南。未来的研究应使用标准化的特定工具来评估EB患者的QoL，同时考虑不同年龄组的特殊性。