%0 Journal Article
%T Papillon-Lefevre Syndrome: A Case Report
%A Sanchita Kundu
%A Sanchita Kundu Shabnam Zahir
%J Oral & Maxillofacial Pathology Journal
%D 2012
%I Kairali Society of Oral & Maxillofacial Pathologists
%X Papillon-Lefevre Syndrome is a very rare syndrome of autosomal recessiveinheritance characterized by palmer-planter hyperkeratosis and early onset of a severedestructive periodontitis., leading to premature loss of both primary and permanentdentitions.Genetic studies have shown that mutations in the major gene locus ofchromosome 11q14 with loss of function of cathepsin C gene are responsible for thissyndrome. An early diagnosis and intervention can help to preserve teeth and preventtheir premature exfoliation. A clinical case of Papillon- Lefevre syndrome isdiscussed herewith having all of the characteristic features.
%K Papillon-Lefevre syndrome
%K palmoplanter hyperkeratosis
%K periodontitis
%U http://a4add.com/remoteupload/uploads/2040/share/13412969411376_myfile.pdf