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Quality of life in people with epidermolysis bullosa: a systematic review

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Abstract

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.

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Funding

This study was funded in part by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior-Brasil (CAPES)-Finance Code 001.

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Authors and Affiliations

  1. Graduate Program in Human Nutrition, Faculty of Health Science, University of Brasília, Campus Universitário Darcy Ribeiro, Brasília, Federal District, 70910-900, Brazil

    C. C. G. Togo, K. M. B. de Carvalho & E. S. Dutra

  2. Clinical Nutrition Unit, University Hospital of Brasília, Brasília, Federal District, Brazil

    A. P. C. Zidorio

  3. Department of Nutrition, Faculty of Health Science, University of Brasília, Brasília, Federal District, Brazil

    V. S. S. Gonçalves

  4. Department of Nutrition and Dietetics, St Thomas’ Hospital, London, UK

    L. Hubbard

Authors
  1. C. C. G. Togo
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  2. A. P. C. Zidorio
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  3. V. S. S. Gonçalves
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  4. L. Hubbard
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  5. K. M. B. de Carvalho
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  6. E. S. Dutra
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Contributions

All authors contributed to the study conception and design. The material preparation, data collection, and analysis were performed by CCGT and APCZ. The first draft of the manuscript was written by CCGT, and all authors commented on previous versions of the manuscript. LH performed the final review with a significant contribution in her area of expertise. All authors read and approved the final manuscript.

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Correspondence to C. C. G. Togo.

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Togo, C.C.G., Zidorio, A.P.C., Gonçalves, V.S.S. et al. Quality of life in people with epidermolysis bullosa: a systematic review. Qual Life Res 29, 1731–1745 (2020). https://doi.org/10.1007/s11136-020-02495-5

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