Papillon-Lefevre Syndrome: A Case Report

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.