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

Research output: Contribution to journalArticle

11 Citations (Scopus)

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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Epidermolysis Bullosa
Blister
Microstomia
Dental Enamel Hypoplasia
Confidence Intervals
Oral Medicine
Inborn Genetic Diseases
Erythema

Cite this

Fortuna, Giulio ; Chainani-Wu, Nita ; Lozada-Nur, Francina ; Aria, Massimo ; Cepeda-Valdes, Rodrigo ; Pollio, Annamaria ; Marinkovich, M. Peter ; Martinez-Salazar, Adriana E. ; Mignogna, Michele D. ; Bruckner, Anna L. ; Salas-Alanís, Julio Cesar. / Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment. In: Journal of the American Academy of Dermatology. 2013 ; pp. 83-92.
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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. 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. {\circledC} 2012 by the American Academy of Dermatology, Inc.",
author = "Giulio Fortuna and Nita Chainani-Wu and Francina Lozada-Nur and Massimo Aria and Rodrigo Cepeda-Valdes and Annamaria Pollio and Marinkovich, {M. Peter} and Martinez-Salazar, {Adriana E.} and Mignogna, {Michele D.} and Bruckner, {Anna L.} and Salas-Alan{\'i}s, {Julio Cesar}",
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Fortuna, G, Chainani-Wu, N, Lozada-Nur, F, Aria, M, Cepeda-Valdes, R, Pollio, A, Marinkovich, MP, Martinez-Salazar, AE, Mignogna, MD, Bruckner, AL & Salas-Alanís, JC 2013, 'Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment', Journal of the American Academy of Dermatology, pp. 83-92. https://doi.org/10.1016/j.jaad.2012.04.009

Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment. / Fortuna, Giulio; Chainani-Wu, Nita; Lozada-Nur, Francina; Aria, Massimo; Cepeda-Valdes, Rodrigo; Pollio, Annamaria; Marinkovich, M. Peter; Martinez-Salazar, Adriana E.; Mignogna, Michele D.; Bruckner, Anna L.; Salas-Alanís, Julio Cesar.

In: Journal of the American Academy of Dermatology, 01.01.2013, p. 83-92.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment

AU - Fortuna, Giulio

AU - Chainani-Wu, Nita

AU - Lozada-Nur, Francina

AU - Aria, Massimo

AU - Cepeda-Valdes, Rodrigo

AU - Pollio, Annamaria

AU - Marinkovich, M. Peter

AU - Martinez-Salazar, Adriana E.

AU - Mignogna, Michele D.

AU - Bruckner, Anna L.

AU - Salas-Alanís, Julio Cesar

PY - 2013/1/1

Y1 - 2013/1/1

N2 - 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.

AB - 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.

U2 - 10.1016/j.jaad.2012.04.009

DO - 10.1016/j.jaad.2012.04.009

M3 - Article

SP - 83

EP - 92

JO - Journal of the American Academy of Dermatology

JF - Journal of the American Academy of Dermatology

SN - 0190-9622

ER -