Abstract
INTRODUCTION: Papillon-Lefèvre 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 "Papillon-Lefèvre syndrome in dentistry," "Etiology of Papillon-Lefèvre syndrome," "Oral manifestations of Papillon-Lefèvre syndrome," "Management of Papillon-Lefèvre syndrome," and "Papillon-Lefèvre syndrome." A total of 47 publications that provided relevant information and discussed the various aspects of PLS were identified. CONCLUSION: The management of PLS necessitates a multidisciplinary approach, including the active involvement of a dental surgeon, dermatologist, and pediatrician to ensure comprehensive care. Extraction of primary teeth and administration of antibiotics is a successful treatment strategy, while placement of removable partial denture is the best option for pediatric patients.