Prophylactic Treatment for Papillon-Lefevre Syndrome with a Four-Year Follow up: A Case Report

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder. Clinical manifestations of this disease are hyperkeratosis of the palms and soles and rapidly progressive periodontitis, which result in premature loss of deciduous and permanent teeth. Early diagnosis and correct treatment of this discover could prevent the involvement of permanent teeth and promote periodontal condition, which leads to longer maintenance of deciduous teeth and prevention of alveolar ridge breakdown and prosthetic problems.
Case report: Our case was a 4-year-old boy presented with PLS, hyperkeratosis of the palms and soles and rapidly progressive periodontitis, which had led to the loosening of deciduous teeth. The case has been followed for four years. Evidence-based treatment of the patient resulted in stabilized periodontal condition and the possibility of partial removable prosthodontics for aesthetic and functional reasons. In addition, periodontal condition of permanent teeth of the patient was favorable after the eruption.
Due to periodontal disease, dentists are often the first to diagnose this syndrome. Early diagnosis and treatment of PLS is of paramount importance due to the possibility of preventing the involvement of permanent teeth.