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Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report
- Source :
- RGO: Revista Gaúcha de Odontologia, Vol 68 (2020)
- Publication Year :
- 2020
- Publisher :
- Faculdade São Leopoldo Mandic, 2020.
-
Abstract
- ABSTRACT Papillon-Lefèvre syndrome is a rare autosomal recessive genetic disorder with a prevalence of one to four cases per million. It is characterized by diffuse or localized palmoplantar hyperkeratosis. It has no gender or racial predilection. Etiology and pathogenesis of Papillon-Lefèvre syndrome are still obscure. However, Papillon-Lefèvre syndrome is believed to be caused by a genetic defect located on chromosome 11, which is responsible for coding cathepsin C, a lysosomal protease present mainly in the epithelial regions such as the palms, soles of feet, knees, and keratinized oral mucosa, which are the most commonly affected areas in Papillon-Lefèvre syndrome. Periodontitis is an oral manifestation of Papillon-Lefèvre syndrome that results in early loss of deciduous and permanent teeth. This report aims to describe a case of an 11-year-old male patient diagnosed with Papillon-Lefèvre syndrome at 4 years of age. After the diagnosis, the child lost all deciduous teeth and sought dental service at the Federal University of Goiás 2 years ago. He is being treated using a multidisciplinary approach involving the areas of periodontics, pathology, and orthodontics to avoid loss of permanent teeth and to provide conditions for preserving the health of remaining teeth while maintaining the function and the esthetics.
- Subjects :
- Papillon-Lefevre disease
Periodontal disease
Tooth loss
Dentistry
RK1-715
Subjects
Details
- Language :
- English
- ISSN :
- 19818637
- Volume :
- 68
- Database :
- Directory of Open Access Journals
- Journal :
- RGO: Revista Gaúcha de Odontologia
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.bc136d3711de4ea084146c54ef89b293
- Document Type :
- article
- Full Text :
- https://doi.org/10.1590/1981-86372020000423635