Papillon Lefevre Syndrome; A Case Report in Two Siblings

Öz

Papillon Lefevre syndrome is an extremely rare syndrome. Since it is seen together with various intraoral signs; dentists play an important role in the diagnosis and treatment of the disease. In this case report, the intraoral and extraoral signs of two sisters who applied to Yüzüncü Yıl University Faculty of Dentistry Department of Oral of Papillon Lefevre syndrome were evaluated and they were directed to the relevant departments for the necessary treatments. Dentists should have information about the intraoral signs and prognosis of Papillon Lefevre syndrome. Thus, the quality of life of patients with Papillon Lefevre syndrome will increase significantly.

Anahtar Kelimeler

• Papillon Lefevre disease
• periodontitis
• premature tooth loss
• hyperkeratosis

Papillon Lefevre Sendromu; Kardeşler Arasında Görülen İki Olgu Sunumu

Öz

Papillon Lefevre sendromu oldukça nadir görülen bir sendromdur. Çeşitli intraoral bulgularla beraber görüldüğünden; hastalığın tanı ve tedavisinde diş hekimlerinin rolü oldukça büyüktür. Bu vaka raporumuzda Yüzüncü Yıl Üniversitesi Diş Hekimliği Fakültesi Ağız, Diş ve Çene Radyolojisi Anabilim Dalı’na Papillon Lefevre sendromu tanısı ile başvuran iki kız kardeşe ait intraoral ve ekstraoral bulgular değerlendirilmiş; gerekli tedavilerin yapılması için ilgili anabilim dallarına yönlendirilmiştir. Diş hekimlerinin Papillon Lefevre sendromunun intraoral bulguları ve prognozu hakkında bilgi sahibi olmaları ve hastalara bu yönde bir tedavi prosedürü uygulamaları hastaların yaşam kalitesini ciddi anlamda artıracaktır.

Anahtar Kelimeler

• Papillon Lefevre Sendromu
• periodontitis
• erken diş kaybı
• hiperkerato

Kaynakça

1. 1. Coeli F, Macedo D, Batista M, Cestari S, Rotta O. Do you know this syndrome? An Bras Dermatol. 2008;83(4):375-377.
2. 2. Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmarplantar hyperkeratosis and premature periodontal destruction of the teeth. J Pediatr. 1964;65:895–908.
3. 3. Aibarrak Z, Alqarni A, Chalisserry E, Sukumaran A. Papillon–Lefèvre syndrome: a series of five cases among siblings. J Med Cases. 2016;10(1):260.
4. 4. Zhang Y, Lundgren T, Renvert S, et al. Evidence of a founder effect for four cathepsin C gene mutations in PapillonLefèvre syndrome patients. J Med Genet. 2001;38(2):96-101.
5. 5. Canger EM, Celenk P, Devrim I, Yenisey M, Gunhan O. Intraoral findings of Papillon-LeFevre syndrome. J Dent Child (Chic). 2008;75(1):99-103.
6. 6. Stabholz A, Taichman NS, Soskolne WA. Occurrence of Actinobacillus actinomycetemcomitans and antileukotoxin antibodies in some members of an extended family affected by PapillonLefèvre syndrome. J Periodontol. 1995;66(7):653-657
7. 7. Serin BA, Doğan MC, Çiftçi V, Özçelik O. Multidisipliner yaklaşım ile papillon lefevre sendromu: 6 yıllık takip. SDÜ Saglik Bilimleri Enstitüsü Dergisi. 2012;3(1):39-44.
8. 8. Dhanrajani PJ. Papillon-Lefevre syndrome: clinical presentation and a brief review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;108(1):e1-e7.

9. 9. Ullbro C, Crossner CG, Nederfors T, Alfadley A, Thestrup-Pedersen K. Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome. J Am Acad Dermatol. 2003;48(3):345-351.
10. 10. Almuneef M, Khenaizan S, Ajaji S, AlAnazi A. Pyogenic liver abscess and Papillon- Lefevre syndrome: Not a rare association. Pediatrics. 2003;111(1):e85- 88.
11. 11. Kanthimathinathan HK, Browne F, Ramirez R, et al. Multiple cerebral abscesses in Papillon-Lefèvre syndrome. Childs Nerv Syst. 2013;29(8):1227-1229.
12. 12. Alpaslan AA, Gül Ü, Akbay G, Karabay Y. Papillon– Lefevre Sendromu: Kardeş İki Olgunun Sunumu. T Klin Dermatoloji. 1995;5:28–32.
13. 13. Pirgon Ö, Atabek ME, Sert A. Papillon– Lefevre Sendromu: Olgu Sunumu. Erciyes Tıp Dergisi. 2007;29(6):478–481.
14. 14. De Haar SF, Tigchelaar–Guter W, Everts V, Beertsen W. Structure of the periodontium in cathepsin C–deficient mice. Eur J Oral Sci. 2006;114(2):171– 173.
15. 15. Özçelik O, Haytaç MC. Papillon–Lefevre Sendromu ile ilişkili periodontitis olgusu: 1 yıllık takip. AÜ Diş Hek. Fak. Derg. 2006;33(1):127–133.
16. 16. Pilger U, Hennies HC, Truschnegg A, Aberer E. Late–onset Papillon–Lefevre syndrome without alteration of the cathepsin C gene. J Am Acad Dermatol. 2003;49(5):240–243.
17. 17. Wara–Aswapati N, Lertsirivorakul J, Nagasawa T, Kawashima Y, Ishikawa I. Papillon–Lefevre syndrome: serum immunoglobulins G (IgG) subclass antibody response to periodontopathic bacteria. A case report. J Periodontol. 2001;72(12):1747– 1754.
18. 18. Kinaia BM, Hope K, Zuhaili A, Tulasne JF. Full-Mouth rehabilitation with calvarium bone grafts and dental ımplants for a Papillon-Lefèvre Syndrome patient: Case Report. Int J Oral Maxillofac Implants. 2017;32(6):e259-e264.
19. 19. Rathod VJ, Joshi NV. Papillon-Lefevre syndrome: A report of two cases. J Indian Soc Periodontol. 2010;14(4):275-278.
20. 20. Idon PI, Olasoji HO, Fusami MA. Papillon-lefevre syndrome: review of literature and report of three cases in the same family. Niger Postgrad Med J. 2015;22(1):75-82.