Papillon Leferve syndrome: A Case Report and Review

Papillon- Lefevre syndrome (PLS) is a rare autosomal recessive disorder of keratinzation characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition.. Its reported incidence is 1–4 per million and both the sexes are equally affected. It is characterized by palmo-plantar hyperkeratosis, periodontopathy and premature loss of deciduous as well as permanent dentition. It manifests between 1–5 year of life and the patient becomes edentulous in the early teens. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes a case of PLS and review of the relevant literature.