| Summary: | Background: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by a diffused palmoplantar hyperkeratosis and severe early-onset periodontitis. Although the exact pathogenesis of this syndrome is still unknown, however, it has been linked to mutations in the cathepsin C gene. Case report: This paper is a clinical presentation of a 12-year-old male with severe periodontitis and characteristic palmoplantar hyperkeratosis diagnosed as PLS. Conclusion: An early diagnosis of the syndrome can help preserve the teeth by the early institution of treatment, using a multidisciplinary approach. Thereby, sparing the patients increase the risk of social, psychological, and economical stigma. Owing to the vast degree of periodontal breakdown involved at such an early age. Incidentally, the dentist is might often be the first to encounter such patients.
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