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).
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
Journal
JAAD international
ISSN: 2666-3287
Titre abrégé: JAAD Int
Pays: United States
ID NLM: 101774762
Informations de publication
Date de publication:
Mar 2021
Mar 2021
Historique:
accepted:
03
12
2020
entrez:
19
8
2021
pubmed:
20
8
2021
medline:
20
8
2021
Statut:
epublish
Résumé
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. 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 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. 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. Both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.
Sections du résumé
BACKGROUND
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
OBJECTIVE
To compare the reliability, convergent validity, and discriminant validity of the EBDASI and iscorEB scoring tools.
METHODS
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
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
CONCLUSIONS
Both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.
Identifiants
pubmed: 34409361
doi: 10.1016/j.jdin.2020.12.007
pii: S2666-3287(20)30084-5
pmc: PMC8362226
doi:
Types de publication
Journal Article
Langues
eng
Pagination
134-152Informations de copyright
© 2020 Published by Elsevier on behalf of the American Academy of Dermatology, Inc.
Déclaration de conflit d'intérêts
Dr Dedee F. Murrell developed the EBDASI. Dr Dedee F. Murrell and Authors Daniel and Su were involved in the validation of the EBDASI. Dr Kern and authors Rogers, Gibson, Martin, Robertson, Feng, and Oliver G. C. Murrell have no conflicts of interest to declare.
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