Serkan DÜNDAR, Mustafa ERSÖZ, Eriş Sinan YÜCEL, Özge ÇELİK, Abubekir ELTAS

Pediatrik Bir Hastada Papillon Lefevre Sendromu ve Oral Bulguları: Nadir Bir Olgu Sunumu

Papillon-Lefevre Sendromu PLS nadir görülen otozomal resesif genetik geçişli bir hastalıktır. Etyolojisinde Cathepsin C Gen mutasyonu sorumlu tutulmaktadır. PLS, palmoplanter keratosis ve yaşamın erken dönemlerinde süt ve daimi dişleri etkileyen şiddetli doku yıkımıyla birlikte görülen periodontitis ile karakterizedir. Bu vakada PLS ve oral bulgularını sunmayı amaçladık

Anahtar Kelimeler:

Papillon-Lefevre sendromu, palmoplanter keratosis, periodontitis, Cathepsin C Geni

Papillon Lefevre Syndrome and Oral Findings in Pediatric Patient: A Rare Case Report

Papillon-Lefevre Syndrome PLS is a rare autosomal recessive disorder. Cathepsin C Gene mutation are responsible for the etiology. PLS characterized by palmoplantar keratosis and severe periodontal destruction in early ages, affecting the primary and permanent dentition. In this case, we aimed to present a rare PLS and its oral findings

Keywords:

Papillon-Lefevre syndrome, palmoplantar keratosis, periodontitis, Cathepsin C Gene,

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1. Uçmak D, Yeşilova Y, Er MN, Dal T. Three cases of Papillon-Lefevre syndrome : Case Report. Journal of Clinical and Experimental Investigations 2011; 2 (2): 222-224.
2. Bicer AZY, Peker I, Demirel O. PapillonLefevre Syndrome: Report of two cases in the same family. Cumhuriyet Dent J 2012;15(2):149-155.
3. Hattab FN, Amin WM. Papillon-Lefe`vre syndrome with albinism: A review of the literature and report of 2 brothers. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:709-716.
4. Dhanrajani PJ. Papillon-Lefevre syndrome: Clinical presentation and a brief review.Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:e1-e7.
5. Ghaffer KA, Zahran FM, Fahmy HM, Brown RS. Papillon-Lefèvre syndrome: neutrophil function in 15 cases fron 4 families in Egypt. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999; 88: 320-325.
6. Wiebe CB, Hakkinen L, Putnins EE, Walsh P, Larjava HS. Successful periodontal maintenance of a case with Papillon-Lefevre syndrome: 12-year follow-up and review of the literature. J Periodontol 2001;72:824-830.
7. Haytaç MC, Özçelik O. Papillon-Lefevre Synydrome Periodontitis: 1 Year Follow-up (A Clinical Report). Journal of Ankara University Faculty of Dentistry. 2006,33(1) 127-133.
8. Allende LM, Moreno A, de Unamuno P. A genetic study of cathepsin C gene in two families with Papillon-Lefèvre syndrome. Mol Genet Metab 2003; 79: 146-148.
9. Etöz OA, Ulu M, Kesim B. Treatment of Patient With Papillon-Lefevre Syndrome With Short Dental Implants: A Case Report. Implant Dent 2010;19:394–399.
10. Yıldırım ZK, Derelioğlu SS, Kantarcı M, Yılmaz Y, Büyükavcı M. Papillon-Lefèvre syndrome: report of three cases in the same family. The Turkish Journal of Pediatrics 2012; 54: 171-176.
11. Nazarro V, Blanchet-Bardon C, Mimoz C, Revuz J, Puis¬sant A. Papillon-Lefevre syndrome. Arch Dermatol 1998;40(6):533-539.
12. Pratchyapruit WO, Kullavavanijaya P. Papillon-Lefevre syn¬drome: a case report. J Dermatol 2002; 29(5):329-335.
13. Judge MR, Mclean WHI, Munro CS. Disorders of Kerati¬nization. Rook’s Textbook of Dermatology. ED. Burns T, Berathnach S, Cox N, Griffiths C. Oxford, Blackwell Pub-lishing 2004;34,111.
14. Woo I, Brunne DP, Yamashita DD, Le BT. Dental implants in a young patient with Papillon-Lefevre syndrome: A case report. Implant Dent. 2003;12:140-144.
15. Nickles K, Schacher B, Ratka-Krüger P, Krebs M, Eickholz P. Long-term results after treatment of periodontitis in patients with Papillon–Lefevre syndrome: success and failure. J Clin Periodontol 2013; 40: 789–798.
16. Pacheco JJ, Coelho C, Salazar F, Contreras A, Slots J, Ve¬lazco CH. Treatment of PapillonLefevre syndrome perio¬dontitis. J Clin Periodontol 2002;29(4):370-374.
17. Rudiger S, Petersilka G, Flemming TF. Combined systemic and local antimicrobial therapy of periodontal disease in PapillonLefevre syndrome. A report of 4 cases. J Clin Periodontol 1999;26(9):847-854.