A comparison study of outcome measures for epidermolysis bullosa: Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and the Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB)

Clare L Rogers; Matthew Gibson; Johannes S Kern; Linda K Martin; Susan J Robertson; Benjamin S Daniel; John C Su; Oliver G C Murrell; Grant Feng; Dedee F Murrell

doi:10.1016/j.jdin.2020.12.007

A comparison study of outcome measures for epidermolysis bullosa: Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and the Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB)

JAAD Int. 2021 Jan 21:2:134-152. doi: 10.1016/j.jdin.2020.12.007. eCollection 2021 Mar.

Authors

Clare L Rogers^{1

2

3}, Matthew Gibson^{1

2

3}, Johannes S Kern^{4

5}, Linda K Martin⁶, Susan J Robertson^{4

7

8

9}, Benjamin S Daniel^{1

2}, John C Su^{7

8

9

10

11}, Oliver G C Murrell³, Grant Feng¹, Dedee F Murrell^{1

2

3}

Affiliations

¹ Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
² Department of Dermatology, St George Hospital, Sydney, New South Wales, Australia.
³ Premier Specialists, Dermatology Trials Australia, Kogarah, New South Wales, Australia.
⁴ Department of Dermatology, The Royal Melbourne Hospital, Parkville, Victoria, Australia.
⁵ Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
⁶ Sydney Children's Hospital, Randwick, New South Wales, Australia.
⁷ Department of Dermatology, The Royal Children's Hospital, Melbourne, Victoria, Australia.
⁸ Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
⁹ Department of Dermatology, Monash Health, Melbourne, Victoria, Australia.
¹⁰ Department of Pediatrics, The University of Melbourne, Melbourne, Victoria, Australia.
¹¹ Department of Medicine, Monash University, Melbourne, Victoria, Australia.

Abstract

Background: 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 but have never been directly compared.

Objective: To compare the reliability, convergent validity, and discriminant validity of the EBDASI and iscorEB scoring tools.

Methods: An observational cohort study was conducted in 15 patients with EB. Each patient was evaluated using the EBDASI and iscorEB-clinician scoring tools by 6 dermatologists with expertise in EB. Quality of life was assessed using the iscorEB-patient and Quality of Life in EB measures.

Results: The intraclass correlation coefficients for interrater reliability were 0.942 for the EBDASI and 0.852 for the iscorEB-clinician. The intraclass correlation coefficients for intrarater reliability was 0.99 for both scores. The two tools demonstrated strong convergent validity with each other.

Conclusion: Both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.

Keywords: BEBS, Birmingham Epidermolysis Bullosa Severity Score; BMD, bone mineral densitometry; DDEB, dominant dystrophic epidermolysis bullosa; EB, epidermolysis bullosa; EBDASI, Epidermolysis Bullosa Disease Activity and Scarring Index; EBS, epidermolysis bullosa simplex; Epidermolysis Bullosa Disease Activity and Scarring Index; ICC, intraclass correlation coefficient; Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa; JEB, junctional epidermolysis bullosa; QOLEB, Quality of Life in Epidermolysis Bullosa score; QoL, quality of life; RDEB, recessive dystrophic epidermolysis bullosa; blistering skin disease; dermatology; epidermolysis bullosa; iscorEB, Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa; outcome measure.