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

Giulio Fortuna, Nita Chainani-Wu, Francina Lozada-Nur, Massimo Aria, Rodrigo Cepeda-Valdes, Annamaria Pollio, M. Peter Marinkovich, Adriana E. Martinez-Salazar, Michele D. Mignogna, Anna L. Bruckner, Julio Cesar Salas-Alanís

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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. © 2012 by the American Academy of Dermatology, Inc.
Original languageEnglish
Pages (from-to)83-92
Number of pages10
JournalJournal of the American Academy of Dermatology
DOIs
Publication statusPublished - 1 Jan 2013
Externally publishedYes

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    Fortuna, G., Chainani-Wu, N., Lozada-Nur, F., Aria, M., Cepeda-Valdes, R., Pollio, A., Marinkovich, M. P., Martinez-Salazar, A. E., Mignogna, M. D., Bruckner, A. L., & Salas-Alanís, J. C. (2013). Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment. Journal of the American Academy of Dermatology, 83-92. https://doi.org/10.1016/j.jaad.2012.04.009