Papillon lefevre syndrome- A literature review and case report

Abstract

Papillon-Lefèvre syndrome (PLS) is an extremely rare genetic disorder. This disorder is mostly observed in children from 1-5 years of age. Alteration of CTSC gene which regulates the production of enzyme cathepsin C is responsible for this syndrome. The common characteristics are palmoplantar keratosis and aggressive form of periodontitis. The patient might become completely edentulous in the absence of appropriate and early treatment. Early lose of teeth in young children may have two important concerns- 1. Pychological impact. 2. Prosthetic rehabilitation. Till date, there is no definitive treatment protocol for PLS cases. This article provides a detailed review about the various possible etiologies, clinical features, differential diagnosis and treatment protocols followed for the periodontal management of PLS. This article reports a Case of Papillon Lefèvre syndrome and its management with long term follow up. Keywords: Periodontitis, Papillon Lefevre syndrome.