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The success of clinical trials in Epidermolysis Bullosa (EB) is dependent upon the availability of a valid and reliable scoring tool that can accurately assess and monitor disease severity. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB) were independently developed and validated against the Birmingham Epidermolysis Bullosa Severity Score (BEBS), but have never been directly compared. Our objective was to compare the reliability, convergent validity and discriminant validity of the EBDASI and iscorEB scoring tools. An observational cohort study was conducted in 15 patients with EB. Each patient was evaluated by six dermatologists with expertise in EB using the EBDASI and iscorEB-clinician scoring tools. Quality of life was assessed using the iscorEB-patient and Quality of Life in EB measures. The intraclass correlation coefficients (ICC) for inter-rater reliability were: EBDASI 0.942 and the iscorEB-clinician 0.852. The ICC for intra-rater reliability was 0.99 for both scores. The two tools demonstrated strong convergent validity with each-other. In conclusion, both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.Copyright © 2021

The success of clinical trials in Epidermolysis Bullosa (EB) is dependent upon the availability of a valid and reliable scoring tool that can accurately assess and monitor disease severity. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB) were independently developed and validated against the Birmingham Epidermolysis Bullosa Severity Score (BEBS), but have never been directly compared. Our objective was to compare the reliability, convergent validity and discriminant validity of the EBDASI and iscorEB scoring tools. An observational cohort study was conducted in 15 patients with EB. Each patient was evaluated by six dermatologists with expertise in EB using the EBDASI and iscorEB-clinician scoring tools. Quality of life was assessed using the iscorEB-patient and Quality of Life in EB measures. The intraclass correlation coefficients (ICC) for inter-rater reliability were: EBDASI 0.942 and the iscorEB-clinician 0.852. The ICC for intra-rater reliability was 0.99 for both scores. The two tools demonstrated strong convergent validity with each-other. In conclusion, both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.Copyright © 2021

Contains fulltext : 229445.pdf (Publisher’s version ) (Closed access)

Contains fulltext : 229445.pdf (Publisher’s version ) (Closed access)

Contains fulltext : 229445.pdf (Publisher’s version ) (Closed access)

This article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.Copyright © 2015 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

This article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.Copyright © 2015 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.