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...

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Main Authors: Sunil Kumar Biraggari, K. Krishna Mohana Reddy, J. Sudhakar, Shiva Shankar Bugude, Rajesh Nichenametla, Mazher Ahmed Hakeem, Swati Reddy Tiyyagura
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Dentistry
Online Access:http://dx.doi.org/10.1155/2015/137381
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author Sunil Kumar Biraggari
K. Krishna Mohana Reddy
J. Sudhakar
Shiva Shankar Bugude
Rajesh Nichenametla
Mazher Ahmed Hakeem
Swati Reddy Tiyyagura
author_facet Sunil Kumar Biraggari
K. Krishna Mohana Reddy
J. Sudhakar
Shiva Shankar Bugude
Rajesh Nichenametla
Mazher Ahmed Hakeem
Swati Reddy Tiyyagura
author_sort Sunil Kumar Biraggari
collection DOAJ
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.
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series Case Reports in Dentistry
spelling doaj-art-6d0a7a217a6d4f93aa55b8761fc596fd2025-02-03T01:24:58ZengWileyCase Reports in Dentistry2090-64472090-64552015-01-01201510.1155/2015/137381137381Papillion-Lefèvre Syndrome: Periodontists’ PerspectiveSunil Kumar Biraggari0K. Krishna Mohana Reddy1J. Sudhakar2Shiva Shankar Bugude3Rajesh Nichenametla4Mazher Ahmed Hakeem5Swati Reddy Tiyyagura6G Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaG Pulla Reddy Dental College & Hospital, IndiaPapillion-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.http://dx.doi.org/10.1155/2015/137381
spellingShingle Sunil Kumar Biraggari
K. Krishna Mohana Reddy
J. Sudhakar
Shiva Shankar Bugude
Rajesh Nichenametla
Mazher Ahmed Hakeem
Swati Reddy Tiyyagura
Papillion-Lefèvre Syndrome: Periodontists’ Perspective
Case Reports in Dentistry
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 lefevre syndrome periodontists perspective
url http://dx.doi.org/10.1155/2015/137381
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