Ocular urface quamou neoplaia in Papillon–Lefevre yndrome

Purpoe To report the occurrence of ocular urface quamou neoplaia (ON) in a patient with Papillon–Lefevre yndrome. Deign Interventional cae report. Method A 14-year-old boy preented with a progreively growing limbal leion in the right eye for 2 month. He had aociated erythematou caly leion on the elbow, knee, and feet with periodontoi uggetive of Papillon–Lefevre yndrome. An exciion biopy of the limbal tumor and a kin biopy wa performed. Reult The limbal tumor howed feature of carcinoma in itu with clear margin. The kin biopy howed epidermal hyperplaia and hyperkeratoi with perivacular infiltrate, conitent with Papillon–Lefevre yndrome. Concluion Ocular urface quamou neoplaia may occur in patient with Papillon–Lefevre yndrome, an aociation that to the bet of our knowledge ha not been previouly reported.