Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score

Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score

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

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

Source

Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research, Stanford, California 94305, USA. giulio.fortuna@gmail.com

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.

Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

PMID:

 

22575158

 

[PubMed – indexed for MEDLINE]

Leave a Comment

Name*

Email* (never published)

Website

Early diagnosis and treatment of oral conditions is critical to maintain your oral and general health.