Papillon Lefevre Syndromewith Multidiciplinar Approach: 6 years follow up

Yıl 2012, Cilt: 3 Sayı: 1, 39 - 44, 31.07.2012

Buse Serin , Muharrem Doğan Volkan Çiftçi , Onur Özçelik,

Öz

This paper presents treatment planning and follows up of 6 year of a child patient with Papillon Lefevre syndrome. Papillon Lefevre syndrome is a rare autosomal recessive syndrome that is characterized by palmoplanar hyperkeratozis and premature loss of tooth. As the result of clinical, immunological and hematological findings, the child patient was diagnosed as having PLS at 2005. The treatment consisting of extraction of all primary teeth at general anesthesia. After an edentulous period complete denture were inserted. And when permanent teeth were erupted, the denture was modified. Treatment procedure was continued with the distal screw with removal appliance. The patient is still under control.

Anahtar Kelimeler

-

Multidisipliner yaklaşım ile Papillon Lefevre Sendromu: 6 yıllık takip

Öz

Papillon Lefevre sendromlu (PLS) bir çocuk hastanın 6 yıllık tedavi ve takip süreci sunulmuştur. Papillon Lefevre sendromu, palmoplantar hiperkeratoz ve dişlerin erken kaybı ile karakterize, nadir görülen otozomal resesif bir hastalıktır. 2005 yılında kliniğimize yönlendirilen çocuk hastaya klinik, immunolojik ve hematolojik bulguların sonucunda hastaya PLS teşhisi konuluştur. Tedavi olarak genel anestezi altında bütün süt dişleri çekilmiştir. Dişsiz periyodu takiben hastaya çocuk protezi uygulaması yapılmıştır. Ve daimi dişleri çıkmaya başladıkça protezi modifiye edilmiştir. Hastada distal vidalı verenli müteharik aparey ile yer açma tedavisine başlanmıştır. Hasta rutin kontrollerine devam etmektedir.

Anahtar Kelimeler

Papillon Lefevre Sendromu, Juvenil

Kaynakça

• Papillon MN, Lefevre B. Two cases of familial sym- metric palmoplanter keratosis (Maleda’s disease) in a brother and his sister. Alterations in both cases (Fren- ch) Bull Soc Francaise Dermatologie Syphiligraphie 1924; 31: 81–84.
• Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmarplantar hyperkeratosis and premature peri- odontal destruction of the teeth. J Pediatr 1964; 65: 895–908.
• Hart TC, Bowden DW, Ghaffar KA, et al. Subloca- lization of the Papillon–Lefevre syndrome locus on 11q14–q21. American Journal of Medical Genetics 1998; 79: 134–139.
• Hart TC, Walker SJ, Bowden DW. An integrated phy- sical and genetic map of the PLS locus interval on chromosome 11q14. Mammalian Genome 2000; 11: 243–246.
• Hart TC, Hart PS, Michalec MD, et al. Localisation of a gene for prepubertal periodontitis to chromosome 11q14 and identification of a cathepsin C gene muta- tion. Journal of Medical Genetics 2000; 37: 95–101.
• Dalgıc B, Bukulmez A, Sarı S. Eponym Papillon–Le- fevre syndrome Eur J Pediatr 2011; 170:689–691.
• Kressin S, Herforth A, Preis S, Wahn V, Lenard HG. Papillon–Lefèvre syndrome – successful treatment with a combination of retinoid and concurrent syste- matic periodontal therapy: case reports. Quintessence International 1995; 26: 795–803
• Lee MR, Wong LCF, Fischer GO. Papillon le–fevre syndrome treated with acitretin. Australasian journal of dermatology. 2005; 46,199–201.
• Haneke E.The Papillon–Lefevre syndrome: kerato- sis palmoplantaris with periodontopathy. Report of a case and review of cases in the literatüre. Hum Ge- net.1979;51:1–35.
• de Haar SF, Tigchelaar–Guter W, Everts V, Beertsen W. Structure of the periodontium in cathepsin C–defi- cient mice. Eur j oral sci 2006; 114: 171–173
• Özçelik O, Haytaç MC. Papillon–Lefevre Sendromu ile ilişkili periodontitis olgusu: 1 yıllık takip. AÜ Diş Hek. Fak. Derg. 2006; 33(1): 127–133.
• Wiebe CB, Hakkinen L, Putnins EE, Walsh P, Lar- java HS. Successful periodontal maintenance of a case with Papillon–Lefevre syndrome: 12–year fol- low–up and review of the literature. J Periodontol 2001;72:824–30.
• Hart TC, Shapira L. Papillon– Lefe`vre syndrome. Periodontol 2000 1994;6:88–100.
• Firatli E, Tuzun B, Efeoglu A. Papillon– Lefe`vre sy- ndrome. Analysis of neutrophil chemotaxis. J Perio- dontol 1996;67:617–20.
• Soskolne WA, Stabholz A, Van Dyke TE, Hart TC & Meyle J. Partial expression of the Papillon–Lefe`vre syndrome in 2 unrelated families. .J Clin Periodontol 1996;23:764–9.
• Lyberg T. Immunological and metabolical studies in two siblings with Papillon– Lefe`vre syndrome. J Pe- riodontol Res 1982;17:563–8.
• Preus HR, Morland B. In vitro studies of monocyte function in two siblings with Papillon– Lefe`vre sy- ndrome. Scand J Dent Res 1987;95:59–64.
• Celenligil H, Kansu E, Ruacan S & Eratalay, K. Pa- pillon–Lefe`vre syndrome. Characterization of perip- heral blood and gingival lymphocytes with monoclo- nal antibodies. J Periodontol 1992;19:392–7.
• Firatli E, Gurel N, Efeoglu A. Papillon– Lefe`vre syndrome. Analysis of peripheral blood lymphocyte subsets. J Clin Periodontol 1996;23:823–25.
• Pacheco JJ, Coelho C, Salazar F, Contreras A, Slo- ts J, Velazco CH. Treatment of Papillon–Lefèvre syndrome periodontitis. J Clin Periodontol. 2002 Apr;29(4):370–4.
• Albandar JM, Khattab R, Monem F, Barbuto SM, Paster BJ. The Subgingival Microbiota of Papillon– Lefèvre Syndrome. J Periodontol. 2011 Dec 5. [Epub ahead of print]
• Colombo AP, Boches SK, Cotton SL, Goodson JM, Kent R, Haffajee AD, Socransky SS, Hasturk H, Van Dyke TE, Dewhirst F, Paster BJ. Comparisons of sub- gingival microbial profiles of refractory periodontitis, severe periodontitis, and periodontal health using the human oral microbe identification microarray. J Peri- odontol. 2009 Sep;80(9):1421–32.
• Robertson KL, Drucker DB, James J, Blinkhorn AS, Hamlet S, Bird PS. A microbiological study of Papil- lon–Lefévre syndrome in two patients. J Clin Pathol. 2001 May;54(5):371–6.
• Pirgon Ö, Atabek ME, Sert A. Papillon–Lefev- re Sendromu: Olgu Sunumu. Erciyes Tıp Dergisi 2007;29(6):478–481
• Gorlin RJ, Cohen MM, Levin LS. Syndromes of the Head and Neck, 3rd edn. Oxford: Oxford University Press, 1990: 853–855.
• Wara–Aswapati N, Lertsirivorakul J, Nagasawa T, Kawashima Y, Ishikawa I. Papillon–Lefevre syndro- me: serum immunoglobulins G (IgG) subclass anti- body response to periodontopathic bacteria. A case report. Journal of Periodontology 2001; 72: 1747– 1754.
• Stabholz A, Taichman S, Soskolne WA. Occurrence of actinobacillus actinomycetemcomitans and anti– leukotoxin antibodies in some members of an exten- ded family affected by Papillon– Lefevre syndrome. J Periodontol 1995; 66: 653–657.
• Eickholz P, Kugel B, Pohl S, Naher H, Staehle HJ. Combined mechanical and antibiotic periodontal the- rapy in a case of Papillon–Lefevre syndrome. J Peri- odontol 2001; 72: 542–549.
• Bani M, Akal N. Papillon Lefevre Sendromu:2 Olgu Sunumu. GÜ Diş Fak Derg 2009;26(2):117–23.
• Siragusa M, Romano C, Batticane N, Batolo D, Sc- hepis C. A new family with Papillon–Lefevre syndro- me: effectiveness of etretinate treatment. Cutis 2000; 65:151–155.
• Dhanrajani PJ. Papillon–Lefevre syndrome: clinical presentation and a brief review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 108(1):1–7.
• Al–Khenaizan S. Papillon–Lefevre syndrome: the response to acitretin. Int J Dermatol 2002; 41:938– 941.
• Lux CJ, Kugel B, Komposch G, Pohl S, Eickholz P. Orthodontic Treatment in a Patient With Papillon– Lefèvre Syndrome. Journal of Periodontology 2005; 76(4): 642–650.
• Bin Dayel AN. Cephalometric findings in patients with Papillon–Lefèvre syndrome. American Journal of Orthodontics & Dentofacial Orthopedics 2008; 134(1): 138–144.
• Toygar HU, Kircelli C, Firat E, Guzeldemir E. Com- bined therapy in a patient with Papillon–Lefèv- re syndrome: a 13–year follow–up. J Periodontol 2007;78(9):1819–24.
• Alpaslan AA, Gül Ü, Akbay G,Karabay Y. Papillon– Lefevre Sendromu: Kardeş İki Olgunun Sunumu. T Klin Dermatoloji 1995; 5: 28–32.
• Uçmak D, Yeşilova Y, Er MN, Dal T. Papillon–lefev- re sendromlu üç olgu. Klinik ve Deneysel Araştırma- lar Dergisi 2011;2(2):222–4.
• Pilger U, Hennies HC, Truschnegg A, Aberer E. Late–onset Papillon–Lefevre syndrome without alte- ration of the cathepsin C gene. J Am Acad Dermatol 2003; 49: 240–243.
• Willett L, Gabriel S, Kozma C, Bottomley WK. Pa- pillon–Lefevre: report of a case. J Oral Med 1985; 40: 43–45.
• Yazdanfar A, Farahnaki S. Late–onset Papillon–Le- fevre syndrome with pyogenic liver abscesses: report of one case. Int J Dermatol. 2009;48(1):76–8.