Publication:
Prevalence of Crown Resorption in Amelogenesis Imperfecta due to Junctional Epidermolysis Bullosa

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dc.contributor.authorBesa, Colomba
dc.contributor.authorOrtega, Ana
dc.contributor.authorVéliz, Sebastián
dc.contributor.authorCornejo, Marco
dc.contributor.authorFuentes, Ignacia
dc.contributor.authorPalisson, Francis
dc.contributor.authorKrämer, Susanne
dc.date.accessioned2026-08-04T21:52:00Z
dc.date.available2026-08-04T21:52:00Z
dc.date.issued2025
dc.description.abstractIntroduction: Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown. Objective: To determine the prevalence of ECR in patients with JEB. Methods: A longitudinal retrospective cohort study was performed at the Special Care Dentistry Clinic, University of Chile. Clinical records of patients with JEB between 2005 and 2024 were analysed. Prevalence of ECR per patient, per type of tooth and per tooth was calculated. Results: Of the 20 patients examined, 90% presented ECR in at least one tooth, with an average of 4.8 primary and 6.8 permanent teeth affected. The most affected type of teeth were the incisors. 57.5% of primary incisors and 68% of permanent incisors had resorption. The most affected tooth was #82 in primary dentition (75%) and #32 in the permanent dentition (88.9%). Conclusions: The prevalence of ECR in patients with AI due to JEB caused by variants in LAMB3 was 90%. Establishing clinical and radiographic dental protocols for the early detection of ECR is essential to prevent extensive tooth destruction.
dc.description.versionVersión Publicada
dc.identifier.citationBesa-Witto C, Ortega-Pinto A, Véliz S, Cornejo M, Fuentes I, Palisson F, Krämer S. Prevalence of Crown Resorption in Amelogenesis Imperfecta due to Junctional Epidermolysis Bullosa. Oral Dis. 2025 Jun;31(6):1900-1908. doi: 10.1111/odi.15250
dc.identifier.doihttps://doi.org/10.1111/odi.15250
dc.identifier.urihttps://hdl.handle.net/11447/10958
dc.language.isoen
dc.subjectLAMB3
dc.subjectamelogenesis imperfecta
dc.subjectcrown resorption
dc.subjectenamel hypoplasia
dc.subjectjunctional epidermolysis bullosa.
dc.titlePrevalence of Crown Resorption in Amelogenesis Imperfecta due to Junctional Epidermolysis Bullosa
dc.typeArticle
dcterms.accessRightsAcceso Abierto
dcterms.sourceOral diseases
dspace.entity.typePublication

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