Papillon Leferve syndrome: A Case Report and Review Dr. Gupta Bhavna, Reader, Dr. Indushekhar KR, Professor & HOD, Dr Sheoran Neha, Senior Lecturer Department of Pedodontics and Preventive Dentistry, Sudha Rustagi Institute of Dental Sciences, Faridabad, Haryana, India Address for Correspondence: Dr. Bhavna Gupta, Reader, Department of Pediatric Dentistry, Sudha Rustagi College of Dental sciences Faridabad, Haryana, India
Online published on 6 March, 2013. Abstract 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. Top Keywords Palmoplantar, Keratosis, recessive disorder, periodontopathy. Top |