Introduction: PapillonLefèvre syndrome (PLS) is an autosomal recessive genetic disorder characterized by the presence of palmoplantar hyperkeratosis on the hands and feet, as well as severe periodontal disease affecting both the primary and permanent teeth, which can lead to premature tooth loss.
Aims: This review aimed to characterize the etiology, clinical manifestations, diagnosis, and recent dental management strategies of pediatric patients with PLS.
Material And Methods: A comprehensive search of the electronic literature was conducted using specific keywords such as "PapillonLefèvre syndrome in dentistry," "Etiology of PapillonLefèvre syndrome," "Oral manifestations of PapillonLefèvre syndrome," "Management of PapillonLefèvre syndrome," and "Papillon-Lefèvre syndrome.