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Puttinger, C., Hecke, G., Strobl, S., Hitthaler-Waigner, P., Welponer, T., Zimmermann, G., Laimer, M., & Salamon, G. (2026). Meta-Analysis of Pain Management in Epidermolysis Bullosa.

British Journal of Dermatology.

Abstract

Pain constitutes one of the most burdensome symptoms in patients with epidermolysis bullosa (EB). Disease heterogeneity, with variations in EB activity, course and extent of systemic involvement, requires a highly individualized approach of analgesic treatment.1 We provide an updated meta-analysis of clinical studies reporting on the scope, efficacy and assessment of analgesic treatment modalities in EB.

This research is reported in accordance with the PRISMA ­guidelines.2 The protocol was registered in PROSPERO (CRD42024601880). Studies between 1991 (when the first consensus paper on EB terminology and classification was published) and 16th October 2024 were included if they reported on the impact of any kind of treatment on EB-associated pain. Retro- and prospective cohort studies, as well clinical trials, were included in the analyses, along with case series of n ≥ 3 patients, while single case reports were excluded due to the potential for a high risk of bias.

Our search strategy yielded a total of 1709 records, among which 352 duplicates were removed. A full search strategy can be found in Appendix S1 at Figshare. Of the remaining 1357 records, 939 (69%) were excluded during title and abstract screening, and another 370 (27%) by full text screening based on eligibility criteria. Thirteen of the remaining 48 studies fulfilled the per protocol requirements to be included in the meta-analysis. However, in four randomized controlled trials (RCTs), missing information was unavailable even on request from the corresponding authors, resulting in nine RCTs being finally included in the meta-analysis. Thereby, a total of nine treatments were investigated, eight local and one systemic, involving 663 patients with EB. Of these studies, 88% (n = 8) were published after the release of the most recent EB pain care guidelines in 2014.3

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