Foot Care in Epidermolysis Bullosa: Evidence-based Guideline

M.T. Khan; M. O'Sullivan; B. Faitli; J.E. Mellerio; R. Fawkes; M. Wood; L.D. Hubbard; A.G. Harris; L. Iacobaccio; T. Vlahovic; L. James; L. Brains; M. Fitzpatrick; K. Mayre-Chilton


The British Journal of Dermatology. 2020;182(3):593-604. 

In This Article

Research Appraisal

All published papers passing this filtering stage were then subjected to a systematic quality appraisal and risk-of-bias assessment. This appraisal was modified from the Critical Appraisal Skills Programme ( and SIGN[36] quality ratings. This allowed both quantitative and qualitative research to be appraised using one list of questions, yielding one quality rating scale to allow a comparison of studies as required (Appendix S8; see Supporting Information). The study limitations and indirectness were taken into account through the appraisal tool. The precision and statistical consistency could not be evaluated as the EB articles had no statistical values. Most studies reviewed had > 50% risk of bias, as EB is a rare condition, there are no double-blind randomized clinical studies and most people would know they have EB.

All selected papers were filtered and appraised by the two panel leads (M.T.K. and M.O'S.). In those instances where consensus could not be reached between the two panel leads, a third appraisal from the panel was allocated until this could be assured. This was conducted to reduce bias, to increase content validity checks of the literature, and most importantly to ensure the consistency of the reviews undertaken. The research quality score was obtained, with a high percentage being indicative of higher quality of the paper. Levels of bias were also measured in percentage values and all papers were graded in accordance with the SIGN method 'Level of Evidence and Grades of Recommendations' 1++ to 4 and grade A to D.[36]

The papers were then divided into outcome topics. All papers and grey literature were allocated to these outcomes. The two panel leads and a member summarized the appraisals per outcome and rated the strength of the recommendation. Outcome summary tables were presented to highlight the population subtypes, numbers of patients, study type, percentage quality and risk of bias in accordance with SIGN. The panel checked the emerging strength of the recommendation, desired and undesired effects, costs related to benefits and the feasibility of implementation. They confirmed and discussed the recommendations elicited using the GRADE framework for the recommendation table ( All recommendation summaries were circulated to the panel, and final agreement and feedback were included. The AGREE II tool[38] was consulted to increase the quality of practice guidelines in rare diseases, and this CPG acknowledges existing guidelines by signposting with the symbol ⇒ throughout this manuscript.

The guidelines were peer reviewed by a representative cross-section of EB multidisciplinary team specialists and people living with EB. Five of eight health professionals and one person living with EB reviewed the guideline draft to assess the degree to which the recommendations presented addressed patients' concerns and identified good practice points (Table S2; see Supporting Information).

The lead and co-lead compiled a reviewer's feedback report for discussion with the guideline panel. Each point was addressed and any resulting change to the guideline was noted or, if no change was made, the reason for this was recorded. The panel conducted a final proofread of the manuscript before submission.