Oral Squamous Cell Carcinoma Associated withPapillon-Lefevre Syndrome: Systematic Review and the First Reported Case

Abstract

Papillon–Lefevre syndrome (PLS) is a rare genodermatosis caused by mutation in CTSC gene which is expressed in epithelial cells as well as in various immune cells including PMNs, macrophages, and their precursors. Such mutation results in palmoplantar hyperkeratosis, early onset periodontitis, susceptibility to infection and rarely mucocutaneous carcinomas. In this study, we aim to perform a systematic review on the incidence of oral squamous cell carcinoma in the association with Papillon-Lefevre syndrome and to introduce the first case report. An electronic search without date and language restrictions in three data bases, Pubmed, Cochrane Library, and Google Scholar in addition to a hand-searching were performed in August 2018. The review of the current literature revealed that such association has not been previously reported. We concluded that patients with Papillon-Lefevre syndrome are susceptible to develop different mucocutaneous carcinomas including OSCC.