Background: Papillon-Lefevre syndrome (PLS) belongs to palmoplantar keratodermas(PPKs). PLS is a rare autosomal recessive disorder characterized by PPKs and keratotic plaques of the elbows and knees as well as periodontal disease with resultant premature loss of deciduous and
permanent teeth.
Case Report: Both patients are male, born and living in Malayer, Iran. There is no history of similar disease in the parents, other siblings and maternal or paternal relatives.
Conclusions: Psoriasiform lesions accompanying palmoplantar keratoderma in patients should leading the physician to examine the mouth for premature loss of teeth and to ask the patient about the family history.

Keywords: Papillon-Lefevre Disease, Keratoderma, Palmoplantar, periodontitis