Papillon-Lefevre Syndrome: A Case Report

PapillonLefevre syndrome (PLS) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset of destructive periodontitis leading to premature loss of both primary and permanent dentition.PLS is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one third of cases. Here we report a case of a 13 year old boy who referred to the Department of Dentistry, Sri Manakula Vinayagar medical college and hospital, Puducherry, India with the complaints of early loss of multiple teeth. He was a first child of healthy consanguineously married parents. On oral examination absence of maxillary and mandibular incisor teeth noted with generalized mobility of all remaining teeth. Hyperkeratosis on the skin of the palms and soles were found. Based on the patient history,clinical and radiographic findings a final diagnosis of PLS was made.