Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: a multicenter development and reliability assessment

J Am Acad Dermatol. 2013 Jan;68(1):83-92. doi: 10.1016/j.jaad.2012.04.009. Epub 2012 May 8.

Abstract

Background: Epidermolysis bullosa (EB) is a genetic mucocutaneous disorder characterized by blister formation upon mild trauma. All 4 EB types may show oropharyngeal lesions involving either hard or soft tissues. Currently, there are very few data on EB scoring that include the oropharyngeal cavity.

Objectives: We sought to develop an oropharyngeal severity score that was objective, valid, reliable, reproducible, easy to perform, and appropriate for all EB types.

Methods: In this study, oral medicine specialists developed a new score, the EB Oropharyngeal Severity (EBOS) score. This measured oropharyngeal disease activity (erythema, atrophy, blisters, erosion/ulceration) and structural damage (microstomia, ankyloglossia, scarring phenotype beyond microstomia and ankyloglossia, enamel hypoplasia). It was tested on 92 patients with different types/subtypes of EB, and interobserver and intraobserver reliability were assessed.

Results: The EBOS mean total score was 12.9 ± 10.9 (range: 0-34). Both interobserver and intraobserver reliability for total score on all patients with EB were considered excellent (intraclass correlation coefficient 0.94; 95% confidence interval 0.90-0.96 and intraclass correlation coefficient 0.90; 95% confidence interval 0.84-0.94, respectively). Even analyzing each single parameter of the disease activity and structural damage, a substantial to excellent correlation was found in the interobserver (except for 4 sites) and intraobserver reliability. A significant correlation was found between EB types/subtypes and the EBOS median score (P < .001), but not between age and the EBOS mean total score in each group.

Limitations: The sample size was small and the number of EB subtypes was limited.

Conclusions: The EBOS score seems to represent an instrument capable of truly quantifying the oropharyngeal severity in different types/subtypes of EB, demonstrating excellent interobserver and intraobserver reliability.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Ankyloglossia
  • Atrophy / etiology
  • Blister / etiology
  • Child
  • Child, Preschool
  • Cicatrix / pathology
  • Confidence Intervals
  • Dental Enamel Hypoplasia / etiology
  • Epidermolysis Bullosa / classification
  • Epidermolysis Bullosa / complications
  • Epidermolysis Bullosa / pathology*
  • Erythema / etiology
  • Female
  • Humans
  • Infant
  • Male
  • Microstomia / pathology
  • Middle Aged
  • Mouth Abnormalities / pathology
  • Mucous Membrane / pathology
  • Observer Variation
  • Oropharynx / pathology*
  • Reproducibility of Results
  • Severity of Illness Index*
  • Statistics, Nonparametric
  • Ulcer / etiology
  • Young Adult