Patterns of oral mucosa lesions in patients with epidermolysis bullosa: comparison and agreement between oral medicine and dermatology

Authors

  • Giulio Fortuna,

    Corresponding author
    1. Department of Oral Medicine, Carolinas Medical Center, Charlotte, NC, USA
    2. D.eb.RA. Mexico Foundation, Monterrey, Nuevo Leon, Mexico
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  • Francina Lozada-Nur,

    1. Department of Orofacial Sciences, School of Dentistry University of California, San Francisco, CA, USA
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  • Annamaria Pollio,

    1. Department of Neurosciences, University of Padua, Padua, Italy
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  • Massimo Aria,

    1. Department of Economics and Statistics, Federico II University of Naples, Naples, Italy
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  • Rodrigo Cepeda-Valdes,

    1. D.eb.RA. Mexico Foundation, Monterrey, Nuevo Leon, Mexico
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  • Matt Peter Marinkovich,

    1. Department of Dermatology, Stanford University School of Medicine, Center for Clinical Sciences Research, Stanford, CA, USA
    2. Division of Dermatology, Veterans Affairs Palo Alto Healthcare System, Palo Alto, CA, USA
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  • Anna L. Bruckner,

    1. Departments of Dermatology and Pediatrics, University of Colorado, School of Medicine, Aurora, CO, USA
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  • Julio Cesar Salas-Alanís

    1. D.eb.RA. Mexico Foundation, Monterrey, Nuevo Leon, Mexico
    2. Dermatology Service, Instituto Tecnológico y de Estudios Superiores de Monterrey, Monterrey, Nuevo Leon, México
    3. Department of Basic Science, Universidad de Monterrey, San Pedro Garza García, Universidad Autonoma de Nuevo Leon, Monterrey, Nuevo Leon, México
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Abstract

Background

The oral mucosa in patients with epidermolysis bullosa (EB) can be affected with different lesions and degrees of severity. However, patterns of oral lesions in distinct types of EB are still unclear.

Objectives

The purpose of this study was to determine the frequency and distribution of four types of lesions (erythema, erosion, atrophy, and blister) for each oral site and to calculate the interobserver reliability for each type of lesion in each site.

Methods

Ninety-two patients with different EB types were assessed independently by an oral medicine specialist and a dermatologist. The degree of agreement was calculated by the intraclass correlation coefficient (ICC).

Results

The most affected oral site was the tongue, with the most frequent lesion being erythema and atrophy [54(58.7%) patients] for the oral medicine specialist and erosion [54(58.7%) patients] for the dermatologist. Patients with recessive dystrophic EB-severe generalized (RDEB-sev gen) showed the highest mean of sites involved by each lesion for both oral medicine and dermatology. The interobserver reliability on the total of lesions was excellent on only 3 sites: lower lip (ICC: 0.89; 95%CI:0.83–0.92), hard palate (ICC:0.85; 95%CI:0.72–0.91), and tongue (ICC:0.89; 95%CI:0.84–0.92), whereas the interobserver reliability calculated for each single oral lesion showed a lower agreement.

Conclusion

Total distribution of sites involved by four types of lesions was higher in RDEB-sev gen than in the rest of EB types, with a predominance of erythema followed by erosion. The agreement on the type of lesion was found to be poor-moderate for many oral sites.

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