Review Paper: Dental Treatment for Patients with Papillon-Lefèvre Syndrome (PLS)

Abstract

Aim: The aim of this article is to present a review of the most current literature regarding the aetiology of the periodontal disease that occurs in patients with Papillon-Lefevre syndrome (PLS). It is also to encourage clinicians to adopt a treatment regime intending to save permanent teeth and alveolar bone in young patients with PLS. Review: The phenotypic expression of Papillon-Lefevre syndrome is heterogeneous as regards the severity of the dermatological as well as the periodontal symptoms. The genetic defect is lack-of-function mutations of the gene encoding for cathepsin C, a lysosomal cysteine proteinase. The defect is associated with a severe reduction in the levels and activities of neutrophil-derived serine proteases (cathepsin G, elastase, Proteinase 3) and in the activation of granzyme B in natural killer cells. About 20% of patients with PLS experience infections besides the periodontal infection, although the patients are not known to be unusually susceptible to viral infections. The reason why many patients with PLS dodevelop periodontal inflammation is most likely due to the fact that the amount of aerobic and anaerobic pathogens in the periodontal pocket overpower a somewhat weakened innate immune response. Based on this literature review a standardised dental treatment protocol for patients with Papillon-Lefevre syndrome is suggested. Conclusion: It is evident that by regularly reducing the periopathogenic flora in the oral cavity and by instigating a potent antibiotic therapy at an early stage of any potential infection, patients with PLS are not predestined to be edentulous in the permanent dentition.