title: Development, reliability, and validity of a novel Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI).
abstract: BACKGROUND
  There is a lack of validated standardized outcome measures for epidermolysis bullosa (EB) that can separate activity from damage.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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 (0.764-0.982). The EBDASI had a higher correlation with PGA (ρ = 0.871) than BEBS with PGA (ρ = 0.852). Intraclass correlation coefficients scatterplots showed the EBDASI was better at distinguishing milder forms of EB, with better correlations at higher severity scores than the BEBS.
  
  
  CONCLUSIONS
  A limited number of patients were recruited for this study. An independent study will be required to demonstrate the responsiveness of the EBDASI.
  
  
  CONCLUSIONS
  The EBDASI demonstrated excellent reliability and validity, as compared with 2 other outcome measures.
authors.affiliation: Department of Dermatology, St George Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Clement C H
authors.initials: CC
authors.lastname: Loh
authors.affiliation: Department of Dermatology, St George Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Jaehwan
authors.initials: J
authors.lastname: Kim
authors.affiliation: Epidermolysis Bullosa Clinic, Royal Children's Hospital, Melbourne, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Australia; Department of Medicine, Monash University, Melbourne, Australia.
authors.forename: John C
authors.initials: JC
authors.lastname: Su
authors.affiliation: Department of Medicine, St Vincent's Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Benjamin S
authors.initials: BS
authors.lastname: Daniel
authors.affiliation: Department of Dermatology, Westmead Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Supriya S
authors.initials: SS
authors.lastname: Venugopal
authors.affiliation: Department of Dermatology, St George Hospital, Sydney, Australia.
authors.forename: Lesley M
authors.initials: LM
authors.lastname: Rhodes
authors.affiliation: Department of Dermatology, St George Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Lizbeth R A
authors.initials: LR
authors.lastname: Intong
authors.affiliation: Kirby Institute, University of New South Wales, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Matthew G
authors.initials: MG
authors.lastname: Law
authors.affiliation: Department of Dermatology, St George Hospital, Sydney, Australia; Faculty of Medicine, University of New South Wales, Sydney, Australia. Electronic address: d.murrell@unsw.edu.au.
authors.forename: Dedee F
authors.initials: DF
authors.lastname: Murrell
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000209:etiology*; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009092:Mucous Membrane / Q000473:pathology; D009262:Nails / Q000473:pathology; D015588:Observer Variation; D015203:Reproducibility of Results; D012535:Scalp / Q000473:pathology; D012720:Severity of Illness Index; D055815:Young Adult
keywords: BEBS; Birmingham Epidermolysis Bullosa Severity; CI; EB; EBDASI; Epidermolysis Bullosa Disease Activity and Scarring Index; ICC; PDAI; PGA; Pemphigus Disease Area Index; Physician Global Assessment; QOL; QOLEB; confidence interval; epidermolysis bullosa; intraclass correlation coefficient; outcome measure; quality of life; quality of life in epidermolysis bullosa; reliability; severity score; validation