Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment - 14/12/12
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.
Le texte complet de cet article est disponible en PDF.Key words : dental, epidermolysis bullosa, oral, prognosis, reliability, score
Abbreviations used : CI, DEB, EB, EBOS, ICC, IQR, JEB, RDEB
Plan
Drs Bruckner and Salas-Alanís equally contributed to the work and need to be considered as last authors. |
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Funding for this work was provided by the Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research; Office of Research and Development, Palo Alto Veterans Affairs Medical Center (Dr Marinkovich); DebRA Mexico Foundation; and Oral Medicine Unit, Department of Odontostomatological and Maxillofacial Sciences, Federico II University of Naples. |
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Conflicts of interest: None declared. |
Vol 68 - N° 1
P. 83-92 - janvier 2013 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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