Development, reliability, and validity of a novel Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI)

Journal of the American Academy of Dermatology
Clement C H LohDédée F Murrell

Abstract

There is a lack of validated standardized outcome measures for epidermolysis bullosa (EB) that can separate activity from damage. We sought to develop and validate an instrument for inherited EB of all ages and subtypes, the EB Disease Activity and Scarring Index (EBDASI), which scores activity responsive to therapy separately from scarring. The EBDASI was validated by comparing its reliability and validity against the Birmingham EB Severity (BEBS) score (partially validated with activity mixed with scarring), using the Physician Global Assessment (PGA) scale as a reference measurement. Sixteen patients with EB (7 EB simplex, 5 dominant dystrophic EB [DDEB], 2 junctional EB, and 2 recessive dystrophic EB) were assessed by 5 EB experts using the EBDASI, BEBS, and PGA, and data from 9 additional patients assessed on an ad hoc basis during routine patient clinic were also included. For interrater reliability, the overall total score intraclass correlation coefficients (95% confidence intervals) were: EBDASI 0.964 (0.929-0.986), BEBS 0.852 (0.730-0.937), and PGA 0.873 (0.765-0.946). For intrarater reliability, the intraclass correlation coefficients were: EBDASI 0.994 (0.976-0.998), BEBS 0.926 (0.748-0.981), and PGA 0.932 (0.764-0....Continue Reading

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Citations

Sep 7, 2013·Orphanet Journal of Rare Diseases·Marilina TampoiaGiuseppina Annicchiarico
Dec 29, 2015·The British Journal of Dermatology·D F Murrell
May 18, 2016·Journal of the American Academy of Dermatology·Joslyn S Kirby
Nov 26, 2018·Journal of the European Academy of Dermatology and Venereology : JEADV·S DănescuR Cosgarea
Aug 10, 2019·The British Journal of Dermatology·M T KhanK Mayre-Chilton
Feb 18, 2020·Journal of the European Academy of Dermatology and Venereology : JEADV·M GibsonD F Murrell
May 24, 2018·Orphanet Journal of Rare Diseases·Brendon W H LeeDedee F Murrell
Jul 12, 2020·Orphanet Journal of Rare Diseases·Christine ProdingerMartin Laimer
Jul 31, 2018·F1000Research·Dimitra Kiritsi, Alexander Nyström
Sep 26, 2020·Nature Reviews. Disease Primers·Ajoy BardhanAdrian H Heagerty
Mar 9, 2021·Journal of the American Academy of Dermatology·Carmen M MontagnonStanislav N Tolkachjov
May 20, 2020·The Journal of Investigative Dermatology·Jemima E Mellerio, Jouni Uitto
Mar 23, 2020·Journal of the American Academy of Dermatology·Victor A EngJean Y Tang

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