title: Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: a multicenter development and reliability assessment.
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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research, Stanford, California 94305, USA. giulio.fortuna@gmail.com
authors.forename: Giulio
authors.initials: G
authors.lastname: Fortuna
authors.forename: Nita
authors.initials: N
authors.lastname: Chainani-Wu
authors.forename: Francina
authors.initials: F
authors.lastname: Lozada-Nur
authors.forename: Massimo
authors.initials: M
authors.lastname: Aria
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Cepeda-Valdes
authors.forename: Annamaria
authors.initials: A
authors.lastname: Pollio
authors.forename: M Peter
authors.initials: MP
authors.lastname: Marinkovich
authors.forename: Adriana E
authors.initials: AE
authors.lastname: Martinez-Salazar
authors.forename: Michele D
authors.initials: MD
authors.lastname: Mignogna
authors.forename: Anna L
authors.initials: AL
authors.lastname: Bruckner
authors.forename: Julio Cesar
authors.initials: JC
authors.lastname: Salas-Alanís
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000072676:Ankyloglossia; D001284:Atrophy / Q000209:etiology; D001768:Blister / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000473:pathology; D016001:Confidence Intervals; D003744:Dental Enamel Hypoplasia / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000145:classification / Q000150:complications / Q000473:pathology*; D004890:Erythema / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008865:Microstomia / Q000473:pathology; D008875:Middle Aged; D009056:Mouth Abnormalities / Q000473:pathology; D009092:Mucous Membrane / Q000473:pathology; D015588:Observer Variation; D009960:Oropharynx / Q000473:pathology*; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D014456:Ulcer / Q000209:etiology; D055815:Young Adult