Papillon-lefèvre syndrome: an unusual case report

Gökhan Özkan, Aydan Kanlı, Saniye Eren Halıcı


Papillon-Lefèvre syndrome (PLS) is a rare autosomal disorder characterized by palmoplantar hyperkeratosis and periodontal problems leading to premature loss of both primary and permanent dentitions. It is caused by mutation of cathepsin C gene generating deficiency in host immune response to microorganisms. Up to date, no sexual or racial predominance has been detected. This paper report the diagnosis and rehabilitation procedures for a 14-year-old male patient with PLS .


Cathepsin C; hyperkeratosis; Papillon-Lefèvre syndrome; prosthetic rehabilitation


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