Translation, cross-cultural adaptation and validation of the Quality of Life Evaluation in Epidermolysis Bullosa instrument in Brazilian Portuguese

Abstract

Background: The Quality of Life Evaluation in Epidermolysis Bullosa (QoLEB) questionnaire was developed for use in English-speaking individuals.

Objectives: The aims of this study were to translate the QoLEB into Brazilian Portuguese, to culturally adapt it, and to verify its reliability and validity.

Methods: The study followed the steps proposed by the World Health Organization, which include: translation; evaluation by a panel of experts and patients; back translation; and linguistic and cultural adaptation. All subjects were examined and assessed using the QoLEB and the Dermatology Life Quality Index (DLQI) or the Children's Dermatology Life Quality Index (CDLQI). Translation and cultural and linguistic adaptation were conducted by 10 patients, three translators, and the bilingual authors.

Results: The population was composed of 40 children (mean age: 8,15 years) and 17 adults (mean age: 33 years). Among the children, eight had epidermolysis bullosa (EB) simplex (EBS), one had junctional EB (JEB), and 31 had dystrophic EB (DEB). In the adult group, four of the subjects had EBS, one had JEB, and 12 had DEB. Mean ± standard deviation (SD) scores on the QoLEB in children were 10.60 ± 7.13 in EBS subjects, 9.71 ± 7.87 in children with dominant DEB (DDEB), and 14.25 ± 9.67 in children with recessive DEB (RDEB). Mean ± SD scores in adults were 12.50 ± 9.95 in EBS subjects, 12.00 ± 5.83 in DDEB subjects, and 20.20 ± 9.21 in RDEB subjects. The QoLEB-BP (Brazilian Portuguese) showed high internal consistency (Cronbach's α = 0.88) and high test-retest reliability (intraclass correlation coefficient: 0.70), confirming the internal consistency and reproducibility of this Portuguese version. There were significant correlations between QoLEB scores and both CDLQI (Pearson's r = 0.688, P < 0.002) and DLQI (Pearson's r = 0.807, P < 0.001) scores.

Conclusions: Epidermolysis bullosa has marked impacts on the lives of EB patients and their families, which are strongly correlated with disease severity. The Brazilian Portuguese version of the QoLEB is validated and can be recommended for use in subsequent studies.