Papillon-Lefevre syndrome: clinical presentation and a brief review.

[papillon-lefèvre syndrome]

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 .