Papillon Lefèvre syndrome: A report of 3 cases with review of literature

Abstract

Papillon Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinisation characterized by palmoplantar hyperkeratosis, periodontopathy and precocious (premature) loss of dentition. Exact mechanism of these clinical events mainly remains speculative. The periodontitis in PLS is usually difficult to control. Effective treatment includes extraction of primary teeth combined with oral antibiotics and professional teeth cleaning. Dentists play a significant role in the diagnosis and management of PLS patients. This paper describes 3 cases of PLS reporting to the out patient department within a span of 2 years with classic clinical features and briefly reviews the literature.

Keywords

• Palmoplantar hyperkeratosis
• papillon Lefèvre syndrome and periodontitis