Papillon-Lefèvre Syndrome- A Case Report and Review

  

  J. Eugenia Sherubin , S. Karthiga Kannan , N. Dhineksh Kumar , Priya Kannan

   DOI : https://doi.org/

 Abstract

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized by hyperkeratosis of the palms and soles with severe destructive periodontitis affecting both primary and permanent teeth, resulting in early tooth loss. Genetic studies have shown that mutations in the major gene locus of chromosome 11q14, with loss of function of the cathepsin C gene, are responsible for PLS. In this report, we describe a case of PLS in a 15-year-old boy and provide a brief review of its clinical presentation, complications, and management.

 Keywords

Papillon-Lefèvre Syndrome, Hyperkeratosis, Periodontitis, Chromosome 11q14, Cathepsin C Gene, Autosomal Recessive Disorder, Early Loss of Teeth.

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