International Journal of Dental Medicine

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

Received: Aug. 28, 2018    Accepted: Sep. 10, 2018    Published: Oct. 10, 2018
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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 OSCC.

DOI 10.11648/j.ijdm.20180402.11
Published in International Journal of Dental Medicine ( Volume 4, Issue 2, December 2018 )
Page(s) 31-35
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Papillon-Lefevre Syndrome, Oral Squamous Cell Carcinoma, Palmoplantar Keratosis, Periodontitis

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. doi: 10.2147/CCIDE.S76080.
[2] Pavankumar K. Papillon–Lefevre syndrome: A case report. The Saudi Dent J 2010; 22: 95-8. doi: 10.1016/j.sdentj.2010.02.009. Epub 2010 Feb 6.
[3] Ragupathy K, Priyadharsini I, Pasupathy S. Parental Consanguinity as a Risk Factor in Papillon-Lefevre Syndrome: A Case Report. Int J Dent Med Res 2015; 1: 68-71. www.ijohmr.com/.../Parental%20Consanguinity%20as%20a%20Risk.
[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. doi: 10.4076/1757-1626-2-7067.
[5] Hacham-Zadeh S, Goldberg L. Malignant melanoma and Papillon-Lefvre syndrome. Arch Dermatol. 1982; 118(1): 2. doi:10.1001/archderm.1982.01650130006003.
[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. Dermatology. 2008; 217(1): 58-62. doi: 10.1159/000124340.
[7] Cook GP. Papillon-Lefèvre syndrome and malignant melanoma. Dermatology. 2009; 219(2):187-8. doi: 10.1159/000224432.
[8] T. Chaubal, R. Bapat and P. Wadkar. Papillon Lefevre syndrome. QJM. 2017; 110: 527, https://doi.org/10.1093/qjmed/hcx090.
[9] Ikeshima A. Papillon-Lefevre syndrome: a highly-suspected case. Journal of oral science 2006; 48: 257-60. https://www.slideshare.net/.../papillonlefevre-syndrome-a-case-report.
[10] Murthy R, Honavar SG, Vemuganti GK, Burman S, Naik M, Parathasaradhi A. Ocular surface squamous neoplasia in Papillon-Lefevre syndrome. Am J Ophthalmol. 2005 Jan; 139(1): 207-9. DOI:10.1016/j.ajo.2004.07.028.
[11] Babu N A, Rajesh E, Krupaa J and Gnananandar G. Genodermatoses. J Pharm Bioallied Sci. 2015; 7: 203–6. DOI: 10.4103/0975-7406.155903.
[12] Ghaffari J, Rezaee S. Gharagozlou M. Chédiak–Higashi syndrome. J Pediatr Rev. 2013; 1: 80-7. http://jpr.mazums.ac.ir/article-1-49-en.html
[13] Bullón P, Castejón-Vega B, Román-Malo L, Jimenez-Guerrero MP, Cotán D, Forbes-Hernandez TY, Varela-López A, Pérez-Pulido AJ, Giampieri F, Quiles JL, Battino M, Sánchez-Alcázar JA, Cordero MD. Autophagic dysfunction in patients with Papillon-Lefèvre syndrome is restored by recombinant cathepsin C treatment. J Allergy Clin Immunol. 2018: S0091-6749(18)30144-1. doi: 10.1016/j.jaci.2018.01.018.
[14] García MSF and Teruya-Feldstein J. The diagnosis and treatment of dyskeratosis congenita: a review. J Blood Med. 2014; 5: 157–67. DOI_1048_Kutbay.
[15] Bouckaert M, Munzhelele TI , Feller L , Lemmer J and Khammissa RAG. The clinical characteristics of oral squamous cell carcinoma in patients attending the Medunsa Oral Health Centre, South Africa. Integr Cancer Sci Therap, 2016; 3: 575-8. DOI: 10.15761/ICST.1000207.
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  • APA Style

    Manar A. Abdul Aziz, Mohamed A. Shawky, Mohammed Atef. (2018). Oral Squamous Cell Carcinoma Associated withPapillon-Lefevre Syndrome: Systematic Review and the First Reported Case. International Journal of Dental Medicine, 4(2), 31-35. https://doi.org/10.11648/j.ijdm.20180402.11

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    ACS Style

    Manar A. Abdul Aziz; Mohamed A. Shawky; Mohammed Atef. Oral Squamous Cell Carcinoma Associated withPapillon-Lefevre Syndrome: Systematic Review and the First Reported Case. Int. J. Dent. Med. 2018, 4(2), 31-35. doi: 10.11648/j.ijdm.20180402.11

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    AMA Style

    Manar A. Abdul Aziz, Mohamed A. Shawky, Mohammed Atef. Oral Squamous Cell Carcinoma Associated withPapillon-Lefevre Syndrome: Systematic Review and the First Reported Case. Int J Dent Med. 2018;4(2):31-35. doi: 10.11648/j.ijdm.20180402.11

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  • @article{10.11648/j.ijdm.20180402.11,
      author = {Manar A. Abdul Aziz and Mohamed A. Shawky and Mohammed Atef},
      title = {Oral Squamous Cell Carcinoma Associated withPapillon-Lefevre Syndrome: Systematic Review and the First Reported Case},
      journal = {International Journal of Dental Medicine},
      volume = {4},
      number = {2},
      pages = {31-35},
      doi = {10.11648/j.ijdm.20180402.11},
      url = {https://doi.org/10.11648/j.ijdm.20180402.11},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.ijdm.20180402.11},
      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 OSCC.},
     year = {2018}
    }
    

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    AB  - 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 OSCC.
    VL  - 4
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Author Information
  • Oral and Maxillofacial Pathology Department, Faculty of Dentistry, Cairo University, Cairo, Egypt

  • Oral and Maxillofacial Surgery Department, Faculty of Dentistry, Cairo University, Cairo, Egypt

  • Oral and Maxillofacial Surgery Department, Faculty of Dentistry, Cairo University, Cairo, Egypt

  • Section