Papillon Lefevre syndrome: A periodontist approach

Introduction: Papillon­Lefevre Syndrome (PLS) is a rare autosomal recessive disorder of keratinisation, characterized by palmoplantar hyperkeratosis, periodontitis and early loss of dentition. Since these features are common to both dentistry and dermatology, members of both disciplines should be aware of the same because an early diagnosis of this condition can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Case Series: The authors here present two cases of Papillon­Lefevre syndrome in siblings, having all of the characteristic features, along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition. Conclusion: The main priority of physician’s strategy is to refer the PLS patients at the earliest to a periodontist for the periodontal management of permanent dentition. Based on the previously reported series and our clinical experience, non­surgical periodontal therapy yielded better results when started immediately after the extraction of deciduous dentition.