A Case Report of Papillon - Lefevre Syndrome

Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder in which there is a palmoplanter keratinisation and premature loss of both deciduous and permanent teeth. Consanguinity of parents is evident in about one third of cases. Earlv diagnosis of the syndrome can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Here we describe a case of Papillon-Lefevre syndrome along with the clinical features, differential diagnosis and management of this syndrome are discussed.