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Papillion-Lefèvre Syndrome: Periodontists' Perspective
Papillion-Lefèvre Syndrome is a very rare disorder of autosomal recessive inheritance distinguished by palmar plantar hyperkeratosis and early onset of periodontitis affecting the dentition. Genetic studies have identified a mutation in the major gene locus of chromosome 11q14 with loss of function....
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4615852/ https://www.ncbi.nlm.nih.gov/pubmed/26523234 http://dx.doi.org/10.1155/2015/137381 |
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author | Biraggari, Sunil Kumar Reddy, K. Krishna Mohana Sudhakar, J. Bugude, Shiva Shankar Nichenametla, Rajesh Hakeem, Mazher Ahmed Tiyyagura, Swati Reddy |
author_facet | Biraggari, Sunil Kumar Reddy, K. Krishna Mohana Sudhakar, J. Bugude, Shiva Shankar Nichenametla, Rajesh Hakeem, Mazher Ahmed Tiyyagura, Swati Reddy |
author_sort | Biraggari, Sunil Kumar |
collection | PubMed |
description | Papillion-Lefèvre Syndrome is a very rare disorder of autosomal recessive inheritance distinguished by palmar plantar hyperkeratosis and early onset of periodontitis affecting the dentition. Genetic studies have identified a mutation in the major gene locus of chromosome 11q14 with loss of function. Cathepsin C gene is to be responsible for Papillion-Lefèvre Syndrome. The present case report describes a 13-year-old female, who visited the Department of Periodontology with the chief compliant of bleeding gums and loose teeth. She presented with the signs and symptoms of Papillion-Lefèvre Syndrome. The patient had premature shedding of her deciduous dentition. On clinical examination, extraorally, the patient presented with persistent thickening, flaking, and scaling of the skin of palms and soles. Her intraoral examination revealed gingival inflammation, abscess formation, and periodontal pockets. Her intraoral radiographs showed bone loss involving the central incisors and molars. The patient underwent periodontal therapy and is under maintenance. |
format | Online Article Text |
id | pubmed-4615852 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-46158522015-11-01 Papillion-Lefèvre Syndrome: Periodontists' Perspective Biraggari, Sunil Kumar Reddy, K. Krishna Mohana Sudhakar, J. Bugude, Shiva Shankar Nichenametla, Rajesh Hakeem, Mazher Ahmed Tiyyagura, Swati Reddy Case Rep Dent Case Report Papillion-Lefèvre Syndrome is a very rare disorder of autosomal recessive inheritance distinguished by palmar plantar hyperkeratosis and early onset of periodontitis affecting the dentition. Genetic studies have identified a mutation in the major gene locus of chromosome 11q14 with loss of function. Cathepsin C gene is to be responsible for Papillion-Lefèvre Syndrome. The present case report describes a 13-year-old female, who visited the Department of Periodontology with the chief compliant of bleeding gums and loose teeth. She presented with the signs and symptoms of Papillion-Lefèvre Syndrome. The patient had premature shedding of her deciduous dentition. On clinical examination, extraorally, the patient presented with persistent thickening, flaking, and scaling of the skin of palms and soles. Her intraoral examination revealed gingival inflammation, abscess formation, and periodontal pockets. Her intraoral radiographs showed bone loss involving the central incisors and molars. The patient underwent periodontal therapy and is under maintenance. Hindawi Publishing Corporation 2015 2015-10-08 /pmc/articles/PMC4615852/ /pubmed/26523234 http://dx.doi.org/10.1155/2015/137381 Text en Copyright © 2015 Sunil Kumar Biraggari et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Biraggari, Sunil Kumar Reddy, K. Krishna Mohana Sudhakar, J. Bugude, Shiva Shankar Nichenametla, Rajesh Hakeem, Mazher Ahmed Tiyyagura, Swati Reddy Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title | Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title_full | Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title_fullStr | Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title_full_unstemmed | Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title_short | Papillion-Lefèvre Syndrome: Periodontists' Perspective |
title_sort | papillion-lefèvre syndrome: periodontists' perspective |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4615852/ https://www.ncbi.nlm.nih.gov/pubmed/26523234 http://dx.doi.org/10.1155/2015/137381 |
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