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Papillon-Lefevre syndrome: clinical presentation and a brief review

Journal
Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontology
Publisher
Elsevier - Mosby
Publication Date
Jan 01, 2009
Volume
108
Issue
1
Identifiers
DOI: 10.1016/j.tripleo.2009.03.016
License
Unknown

Abstract

The Papillon-Lefevre syndrome (PLS) was first described by Papillon and Lefevre in 1924. It is an autosomal recessive disorder characterized by a diffuse palmoplanter hyperkeratosis and rapidly progressive and devastating periodontitis, affecting the primary as well as the permanent dentition, attributed to a point mutation of the cathepsin C gene. This paper presents a clinical presentation and a brief review of its etiology and treatment modalities.

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