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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.