Papillon–Lefèvre syndrome: clinical presentation and management options
Identifieur interne : 000E55 ( Main/Merge ); précédent : 000E54; suivant : 000E56Papillon–Lefèvre syndrome: clinical presentation and management options
Auteurs : Basapogu Sreeramulu [Inde] ; Naragani Dvn Shyam [Inde] ; Pilla Ajay [Inde] ; Pathipaka Suman [Inde]Source :
- Clinical, Cosmetic and Investigational Dentistry [ 1179-1357 ] ; 2015.
Abstract
Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.
Url:
DOI: 10.2147/CCIDE.S76080
PubMed: 26203280
PubMed Central: 4507741
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<front><div type="abstract" xml:lang="en"><p>Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.</p>
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