Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report
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 syndro...
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| Format: | Article |
| Language: | English |
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Faculdade São Leopoldo Mandic
2020-09-01
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| Series: | RGO: Revista Gaúcha de Odontologia |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1981-86372020000100812&tlng=en |
| _version_ | 1797792777929490432 |
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| author | Paolla Leão MARTINS Francine do Couto Lima MOREIRA Cerise de Castro CAMPOS Virgílio Moreira RORIZ |
| author_facet | Paolla Leão MARTINS Francine do Couto Lima MOREIRA Cerise de Castro CAMPOS Virgílio Moreira RORIZ |
| author_sort | Paolla Leão MARTINS |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-13T02:37:42Z |
| format | Article |
| id | doaj.art-bc136d3711de4ea084146c54ef89b293 |
| institution | Directory Open Access Journal |
| issn | 1981-8637 |
| language | English |
| last_indexed | 2024-03-13T02:37:42Z |
| publishDate | 2020-09-01 |
| publisher | Faculdade São Leopoldo Mandic |
| record_format | Article |
| series | RGO: Revista Gaúcha de Odontologia |
| spelling | doaj.art-bc136d3711de4ea084146c54ef89b2932023-06-28T23:14:30ZengFaculdade São Leopoldo MandicRGO: Revista Gaúcha de Odontologia1981-86372020-09-016810.1590/1981-86372020000423635Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case reportPaolla Leão MARTINShttps://orcid.org/0000-0002-5146-1785Francine do Couto Lima MOREIRAhttps://orcid.org/0000-0002-4950-1864Cerise de Castro CAMPOShttps://orcid.org/0000-0003-2156-3315Virgílio Moreira RORIZhttps://orcid.org/0000-0003-2028-164XABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1981-86372020000100812&tlng=enPapillon-Lefevre diseasePeriodontal diseaseTooth loss |
| spellingShingle | Paolla Leão MARTINS Francine do Couto Lima MOREIRA Cerise de Castro CAMPOS Virgílio Moreira RORIZ Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report RGO: Revista Gaúcha de Odontologia Papillon-Lefevre disease Periodontal disease Tooth loss |
| title | Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report |
| title_full | Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report |
| title_fullStr | Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report |
| title_full_unstemmed | Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report |
| title_short | Multidisciplinary dental treatment for Papillon-Lefèvre syndrome: case report |
| title_sort | multidisciplinary dental treatment for papillon lefevre syndrome case report |
| topic | Papillon-Lefevre disease Periodontal disease Tooth loss |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1981-86372020000100812&tlng=en |
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