Scoring dataset for validation of scoring tools for Epidermolysis Bullosa Acquisita (EBA): EBA-IGA, EBA-VIIS, EBDASI & EBADAI
Description
This is the anonymous dataset for the validation of an Investigator Global Assessment (IGA) scale for Epidermolysis Bullosa Acquisita (EBA) (EBA-IGA), as well as the validation of an EBA Visual Impression IGA Scale (EBA-VIIS), the Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and the Epidermolysis Bullosa Acquisita Disease Area Index (EBADAI). 24 international dermatologists participated in this photographic scoring exercise.
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Steps to reproduce
1. Obtain ethics approval for a multi-centre, image-based validation study. 2. Recruit clinical photographs of EBA patients from international centres, including only dermatologist-confirmed EBA with high-quality images, and exclude poor-quality or confounding cases. Include a variety of severities, Fitzpatrick skin types and EBA sub-types. 3. Compile a printed photo booklet of 35 curated, de-identified cases for scoring. 4. Recruit blistering disease experts (dermatologists), approximately 24 globally, to independently score each case using: EBA-VIIS, EBA-IGA (D, P, F sub-scores), EBDASI and EBADAI in a secure, online scoring booklet. Raters should be instructed not to revise EBA-VIIS after completing EBA-IGA. 5. Collect scores via individualised online booklets. 6. Analyse data using SPSS/Excel; calculate means and standard deviations of each scoring tool, interrater ICCs (two-way random, absolute agreement) and convergent validity using Pearson correlation between total activity and overall scores.
Institutions
- University of New South Wales