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Oral Squamous Cell Carcinoma associated with Papillon-Lefevre Syndrome: Systematic Review and the first reported case
EP31325
Poster Title: Oral Squamous Cell Carcinoma associated with Papillon-Lefevre Syndrome: Systematic Review and the first reported case
Submitted on 17 Feb 2020
Author(s): Manar A. Abdul Aziz1, *, Mohamed A. Shawky2, Mohammed Atef2
Affiliations: 1Oral and Maxillofacial Pathology Department, Faculty of Dentistry, Cairo University, Cairo, Egypt 2Oral and Maxillofacial Surgery Department, Faculty of Dentistry, Cairo University, Cairo, Egypt
This poster was presented at annual scientific day, Oral and Maxillofacial Pathology. Faculty of Dentistry, Cairo University
Poster Views: 238
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Poster Information
Abstract: Papillon–Lefèvre 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 OSCCSummary: Papillon–Lefèvre syndrome is a rare genodermatosis that may be associated with mucocutaneous carcinomas. We performed a systematic review on the incidence of OSCC in the association with this syndrome and introduced first case report as such association was not previously reported. A thirty-six years old male patient was complaining of painful palatal ulcerated swelling appeared 25 days ago. Excisional biopsy with safety margins was performed and the lesion was diagnosed as OSCC.References: [1] Sreeramulu B, Shyam N, Ajay P and Suman P. Papillon–Lefèvre syndrome: clinical presentation and management options.Clin Cosmet Investig Dent. 2015; 7: 75–81.
[2] Pavankumar K. Papillon–Lefevre syndrome: A case report. The Saudi Dent J 2010; 22: 95-8.
[3] Ragupathy K, Priyadharsini I, Pasupathy S. Parental Consanguinity as a Risk Factor in Papillon-Lefevre Syndrome:A Case Report. Int J Dent Med Res2015;1:68-71
[4] Sammy Al-Benna,1 Raphael Hasler,1 Ingo Stricker,2 Hans-Ulrich Steinau,1 and Lars Steinstraesser. Papillon-Lefèvre syndrome and squamous cell carcinoma: a case report. Cases J. 2009; 2: 7067.
[5] Hacham-Zadeh S, Goldberg L. Malignant melanoma and Papillon-Lefvre syndrome. Arch Dermatol. 1982; 118(1): 2.
[6] Nakajima K, Nakano H, Takiyoshi N, Rokunohe A, Ikenaga S, Aizu T, Kaneko T, Mitsuhashi Y, Sawamura D. Papillon Lefèvre syndrome and malignant melanoma. A high incidence of melanoma development in Japanese palmoplantar keratoderma patients. Dermat
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