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Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score: A multicenter development and reliability assessment - 14/12/12

Doi : 10.1016/j.jaad.2012.04.009 
Giulio Fortuna, DMD, PhD a, b, d, , Nita Chainani-Wu, DDS, PhD, MPH e, Francina Lozada-Nur, DDS, MS, MPH e, Massimo Aria, PhD c, Rodrigo Cepeda-Valdes, MD d, Annamaria Pollio, DMD b, M. Peter Marinkovich, MD a, f, Adriana E. Martinez-Salazar, MD d, Michele D. Mignogna, MD, DMD b, Anna L. Bruckner, MD g, Julio Cesar Salas-Alanís, MD d, h, i
a Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research, Stanford, California 
b Oral Medicine Unit, Department of Odontostomatological and Maxillofacial Sciences, Federico II University of Naples, Naples, Italy 
c Department of Mathematics and Statistics, Federico II University of Naples, Naples, Italy 
d DebRA (Dystrophic Epidermolysis Bullosa Research Association) Mexico Foundation, Monterrey, Mexico 
e Department of Orofacial Sciences, School of Dentistry, University of California, San Francisco, California 
f Division of Dermatology, Veterans Affairs Palo Alto Healthcare System, Palo Alto, California 
g Departments of Dermatology and Pediatrics, University of Colorado, School of Medicine, Aurora, Colorado 
h Dermatology Service, Instituto Tecnológico y de Estudios Superiores de Monterrey, Monterrey, Mexico 
i Department of Dermatology, Facultad de Medicina y Hospital Universitario Dr José E. González, Universidad Autonoma de Nuevo Leon, Monterrey, Mexico 

Reprint requests: Giulio Fortuna, DMD, PhD, Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research, Marinkovich Laboratory, 269 Campus Dr, Stanford, CA 94305.

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.
 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.
 Conflicts of interest: None declared.


© 2012  American Academy of Dermatology, Inc.. Publié par Elsevier Masson SAS. Tous droits réservés.
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P. 83-92 - janvier 2013 Retour au numéro
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