Papillon-Lefevre Syndrome: A Rare Case Report in Jazan, Kingdom of Saudi Arabia

Abstract

The PapillonLefevre syndrome is an autosomal recessive conditiondescribed by several dermatological manifesta­tions andoral conditions such as periodontitis. Its aetiopathogenesis is thought to be secondary to the mutation of the Cathepsin C gene. It presents with dermatologic feature of Palmoplan­tar keratosis which ranges from a mild psoriasiform scaly skin to overt hyperkeratosis and also affect elbows and knees

This case report presents with an oral manifestations of PapillonLefevre syndrome in a young boy with missing several teeth, which was rehabilated with removable partial denture.