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Papillon-Lefevre Synydrome Periodontitis: 1 Year Follow-up A Clinical Report

Year 2006, Volume: 33 Issue: 2 - Volume: 33 Issue: 2, 127 - 133, 01.10.2006

Abstract

Papillon-Lefevre Syndrome PLS is a rare autosomal recessive disease with a very low prevalence. Characteristic symptoms are diffuse palmar and plantar hyperkeratosis combined with a rapidly progressive periodontal breakdown affecting both dentitions, resulting in premature loss of deciduous and permanent teeth. This paper reports the treatment planning of the severely affected periodontal component of a patient with Papillon-Lefevre Syndrome. Clinical examination which included conventional periodontal measurements and radiographic analysis showed severe inflamation and bone loss. Total blood count, neutrophil function tests and alkaline phosphatase, CD 2, 3, 4, 5, 8, 11b, 16, HLA-DR and immunoglobulin analysis were made. Occurence of major suspected periodontopathic bacteria which included Porphyromonas gingivalis, Bacteriodes forsythus and Trepenoma denticola was determined by BANA N-benzoyl-DL-argininenaphthylamide test. As the result of clinical, immunological, microbiological and hematological findings, the patient was diagnosed as having PLS. The treatment consisted of extraction of all primary teeth and systemic antibiotic therapy. After an edentulous period complete dentures were inserted 3 months after the extractions. The patient is still under control after one year

References

  • Papillon MM, Lefe`vre P. 2 cases of sym- metrically famillary palmar and plantar hypercerato- sis (Meledas didease) within brother and sister com- bined with severe dental alterations in both cases (in French). Soc Franc Dermat Syph 1924;31:82-4.
  • Gorlin RJ, Cohen MM Jr., Levin LS. Syndromes of the head and neck. New York: Oxford University Press, 1990; 853-5.
  • 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;72:824-30.
  • Hart TC, Stabholz A, Meyle J, Shapira L, Van Dycke TE, Cutler CW & Soskolne WA. Genetic studies of syndromes with severe periodontitis and palmoplantar hyperkeratosis. J Periodontal Res 1997;32: 81-9.
  • Bimstein E, Lustmann J, Sela MN, Ben Neriah Z & Soskolne WA. Periodontitis associated with Papillon-Lefe`vre syndrome. J Periodontol 1990;61:373-77.
  • Hart TC, Hart PS, Bowden DW, Michalec MD, Callison SA, Walker SJ, Zhang Y & Firatli E. Mutations of the cathepsin C gene are responsible for Papillon- Lefe`vre syndrome. J Med Genet 1999;36: 881-7.
  • Fischer J, Blanchet-Bardon C, Prud’homme JF, Pavek S, Steijlen PM, Dubertret L & Weissenbach J. Mapping of Papillon-Lefe`vre syn- drome to the chromosome 11q14 region. Eur J Hum Genet 1997;5:156-60.
  • Laass MW, Hennis HC & Preis S, et al. Localization of a gene for Papillon -Lefe`vre syn- drome to chromosome 11q14-q21 by homozygosity mapping. Hum Genet 1997;101:376-82.
  • Fıratlı E, Gurel N, Efeoglu A, Badur S. Clinical and immunological findings in 2 siblings with Papillon- Lefe`vre syndrome. J Periodontol 1996; 67: 1210-5.
  • Nitta H, Kato H, Umeda M, Kuwata S & Ishikawa I. Papillon-Lefe`vre syndrome: analysis of HLA antigens. Oral Dis 2000; 6:278-81.
  • Lundgren T, Renvert S: Periodontal treat- ment of patients with Papillon–Lefe`vre syndrome: a 3-year follow-up. J Clin Periodontol 2004; 31: 933- 8.
  • Hart TC & Shapira L. Papillon- Lefe`vre syndrome. Periodontol 2000 1994;6:88-100.
  • Haneke E, Hornstein OP, Lex C. Increased susceptibility to infections in Papillon-Lefevre syn- drome. Dermatologica 1975;150:283-6.
  • Kleinfelder JW, Topoll HH, Preus HR, Muller RF, Lange DE & Bocker W. Microbiological and immunohistological findings in a patient with Papillon-Lefe`vre syndrome. J Clin Periodontol 1996;23:1032-8.
  • Lundgren T, Renvert S, Papapanou PN & Dahle´n G. Subgingival microbial profile of Papillon-Lefe`vre patients assessed by DNA-probes. J Clin Periodontol 1998;25:624-9.
  • Firatli E, Tuzun B & Efeoglu A. Papillon- Lefe`vre syndrome. Analysis of neutrophil chemo- taxis. J Periodontol 1996;67:617-20.
  • Van Dyke TE, Taubman MA, Ebersole JL, Haffajee AD, Socransky SS, Smith DJ & Genco RJ. The Papillon-Lefe`vre syndrome: neutrophil dys- function with severe periodontal disease. Clin Immunol Immunopathol 1984;31:419-29.
  • Schroeder HE, Seger RA, Keller HU & Rateitschak-Plu¨ ss EM. Behavior of neutrophilic granulocytes in a case of Papillon-Lefe`vre synd- rome. J Clin Periodontol 1983;10:618-35.
  • Stalder JF, Gellard P, Vergracht A, Bignon JD, Dreno B & Litoux P. Abnormalities in the expression of polymorphonuclear adhesion glyco- proteins after stimulation in two patients with Papillon- Lefe`vre syndrome. Card Grenoble Proceedings. J Invest Dermatol 1988;92: 146-51.
  • Umeda M, Zhang VJ, Kosaki T & Ishikawa I. Clinical, bacteriological and immunological exa- mination and treatment of two Papillon-Lefe`vre syndrome patients. J Oral Pathol 1990;57: 430-40.
  • Van Dyke TE, Taubman MA, Ebersole JL, Haffajee AD, Socransky SS, Smith DJ & Genco RJ. The Papillon- Lefe`vre syndrome: Neutrophil dys- function with severe periodontal disease. Clin Immunol Immunopathol 1984;31:419-29.
  • 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 synd- rome. J Periodontol Res 1982;17:563-8.
  • Preus HR & Morland B. In vitro studies of monocyte function in two siblings with Papillon- Lefe`vre syndrome. Scand J Dent Res 1987;95:59- 64.
  • Celenligil H, Kansu E, Ruacan S & Eratalay, K. Papillon-Lefe`vre syndrome. Characterization of peripheral blood and gingival lymphocytes with monoclonal 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.
  • Jung J, Carranza FA & Newman MG. Scanning electron-microscopy of plaque in Papillon- Lefe`vre syndrome. J Periodontol 1981;52:442-46.
  • Newman M, Angel I, Karge H, Weiner M, Grinenko V & Schusterman L. Bacterial studies of the Papillon-Lefe` vre Syndrome. J Dent Res 1977;56:545-46.
  • Preus HR Treatment of rapidly destructive periodontitis in Papillon-Lefe`vre syndrome. Laboratory and clinical observations. J Clin Periodontol 1988;15:639-43.
  • Eronat N, Ucar F & Kilnic G. Papillon- Lefe`vre syndrome: treatment of 2 cases with a cli- nical and microbiological investigation. J Clin Periodontol 1993;17: 99-104.
  • Ishikawa I, Umeda M & Laosrisin N. Clinical, bacteriological and immunological exami- nations and treatment process of two Papillon- Lefe`vre syndrome patients. J Periodontol 1994;65:364-71.
  • Tinanoff N, Tempro P & Maderazo EG. Dental treatment of Papillon-Lefe`vre syndrome: 15- year follow-up. J Clin Periodontol 1995;22:62-86.
  • De Vree H, Steenackers K, De Boever JA. Periodontal treatment of rapid progressive periodon- titis in 2 siblings with papillon-lefevre syndrome: 15- year follow up. J Clin Periodontol 2000;27:354-60.
  • Velazco CH, Coelho C, Salzar F, Contreras A, Slots J & Pacheco JJ. Microbiological features of Papillon–Lefe`vre syndrome periodontitis. J Clin Periodontol 1999;26:622-27.
  • Clerehugh V, Drucker DB, Seymour GJ & Bird PS. Microbiological and serological investiga- tion of oral lesions in Papillon–Lefe`vre syndrome. J Clin Pathol 1996;49:255-7.
  • Baer PN, McDonald RE. Suggested mode of periodontal therapy for patients with Papillon- Lefevre syndrome. Periodontol Case Rep 1981;1:10- 4.
  • Tinanoff N, Tanzer J, Kornman KS & Maderazo EG. Treatment of the periodontal compo- nent of Papillon-Lefe`vre syndrome. J Clin Periodontol 1986;13: 6-10.
  • Lu HKJ, Lin CT & Kwan HW. Treatment of a patient with Papillon- Lefe`vre syndrome: a case report. J Periodontol 1987;56:789-93.
  • Tinanoff N, Tanzer JM, Kornman KS & Maderazo EG. Treatment of the periodontal compo- nent of Papillon-Lefe`vre syndrome: A case report. J Clin Periodontol 1990;61:373-7.
  • Rüdiger S, Petersilka G, Flemmig TF. Combined systemic and local antimicrobial therapy of periodontal disease in Papillon-Lefevre syndrome. A report of 4 cases. J Clin Periodontol 1999;26:847- 54.
  • Lundgren T Crossner CG, Twetman S, Ulbro C. Systemic retinoid medication and periodon- tal health in patients with Papillon-Lefevre syn- drome. J Clin Periodontol 1996; 23:176-9.
  • Kressin S, Herforth A, Preis S, Wahn V & Lenard HG. Papillon-Lefe`vre syndrome-successful treatment with a combination of retinoid and concur- rent systematic periodontal therapy: Case reports. Quintessence Int 1995;26:795- 803.
  • Gelmetti C, Nazzaro V, Cerri D, Fracasso L. Long-term preservation of permanent teeth in a patient with Papillon-Lefevre syndrome treated with etretinate. Pediatr Dermatol 1989;6:222-5.
  • Gorlin RJ, Sedano H, Anderson VE, The syndrome of palmar-plantar hyperkeratosis and pre- mature periodontal destruction of the teeth. J Pediatr 1964;65:895-908.

PAPILLON-LEFEVRE SENDROMU İLE İLİŞKİLİ PERİODONTİTİS OLGUSU: 1 YILLIK TAKİP

Year 2006, Volume: 33 Issue: 2 - Volume: 33 Issue: 2, 127 - 133, 01.10.2006

Abstract

Papillon-Lefevre sendromu, PLS çok nadirgözlenen otozomal resesif bir hastalıktır. Süt vedaimi dişlerde erken kayıplar, hızlı ilerleyen periodontal doku kayıpları ile birlikte gözlenen, avuç içive ayak tabanında yaygın hiperkeratotik alanlarınvarlığı karakteristik bulgularındandır. Bu vaka raporu, PLS’ye bağlı olarak ileriderecede etkilenmiş periodontal dokularındeğerlendirilmesini amaçlamıştır. Rutin periodontal ölçümleri ve radyografik değerlendirmeleri kapsayan klinik incelemelerde, şiddetli inflamasyon vekemik kaybı tespit edilmiştir. Hastaya tam kansayımı, nötrofil fonksiyon testi, alkalen fosfataz,CD2,3,4,5,8,11b,16, HLA-DR ve immünglobulinanalizleri yapılmıştır. Ayrıca Porphyromonas gingivalis, Bacteriodes forsythus ve Trepenoma denticola’yı kapsayan periodonto-patojenler BANA Nbenzoyl-DL-arginine-naphthylamide testi ile tespitedilmiştir. Klinik, immünoljik, mikrobiyolojik vehematolojik bulguların sonucunda, hastaya PLSteşhisi konmuştur. Tedavi olarak, sistemik antibiyotik uygulaması altında bütün dişlerin çekimigerçekleştirilmiştir. Dişsiz periodu takiben 3 aysonra hastaya tam protez uygulaması yapılmıştır vehasta halen kontrol altındadır

References

  • Papillon MM, Lefe`vre P. 2 cases of sym- metrically famillary palmar and plantar hypercerato- sis (Meledas didease) within brother and sister com- bined with severe dental alterations in both cases (in French). Soc Franc Dermat Syph 1924;31:82-4.
  • Gorlin RJ, Cohen MM Jr., Levin LS. Syndromes of the head and neck. New York: Oxford University Press, 1990; 853-5.
  • 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;72:824-30.
  • Hart TC, Stabholz A, Meyle J, Shapira L, Van Dycke TE, Cutler CW & Soskolne WA. Genetic studies of syndromes with severe periodontitis and palmoplantar hyperkeratosis. J Periodontal Res 1997;32: 81-9.
  • Bimstein E, Lustmann J, Sela MN, Ben Neriah Z & Soskolne WA. Periodontitis associated with Papillon-Lefe`vre syndrome. J Periodontol 1990;61:373-77.
  • Hart TC, Hart PS, Bowden DW, Michalec MD, Callison SA, Walker SJ, Zhang Y & Firatli E. Mutations of the cathepsin C gene are responsible for Papillon- Lefe`vre syndrome. J Med Genet 1999;36: 881-7.
  • Fischer J, Blanchet-Bardon C, Prud’homme JF, Pavek S, Steijlen PM, Dubertret L & Weissenbach J. Mapping of Papillon-Lefe`vre syn- drome to the chromosome 11q14 region. Eur J Hum Genet 1997;5:156-60.
  • Laass MW, Hennis HC & Preis S, et al. Localization of a gene for Papillon -Lefe`vre syn- drome to chromosome 11q14-q21 by homozygosity mapping. Hum Genet 1997;101:376-82.
  • Fıratlı E, Gurel N, Efeoglu A, Badur S. Clinical and immunological findings in 2 siblings with Papillon- Lefe`vre syndrome. J Periodontol 1996; 67: 1210-5.
  • Nitta H, Kato H, Umeda M, Kuwata S & Ishikawa I. Papillon-Lefe`vre syndrome: analysis of HLA antigens. Oral Dis 2000; 6:278-81.
  • Lundgren T, Renvert S: Periodontal treat- ment of patients with Papillon–Lefe`vre syndrome: a 3-year follow-up. J Clin Periodontol 2004; 31: 933- 8.
  • Hart TC & Shapira L. Papillon- Lefe`vre syndrome. Periodontol 2000 1994;6:88-100.
  • Haneke E, Hornstein OP, Lex C. Increased susceptibility to infections in Papillon-Lefevre syn- drome. Dermatologica 1975;150:283-6.
  • Kleinfelder JW, Topoll HH, Preus HR, Muller RF, Lange DE & Bocker W. Microbiological and immunohistological findings in a patient with Papillon-Lefe`vre syndrome. J Clin Periodontol 1996;23:1032-8.
  • Lundgren T, Renvert S, Papapanou PN & Dahle´n G. Subgingival microbial profile of Papillon-Lefe`vre patients assessed by DNA-probes. J Clin Periodontol 1998;25:624-9.
  • Firatli E, Tuzun B & Efeoglu A. Papillon- Lefe`vre syndrome. Analysis of neutrophil chemo- taxis. J Periodontol 1996;67:617-20.
  • Van Dyke TE, Taubman MA, Ebersole JL, Haffajee AD, Socransky SS, Smith DJ & Genco RJ. The Papillon-Lefe`vre syndrome: neutrophil dys- function with severe periodontal disease. Clin Immunol Immunopathol 1984;31:419-29.
  • Schroeder HE, Seger RA, Keller HU & Rateitschak-Plu¨ ss EM. Behavior of neutrophilic granulocytes in a case of Papillon-Lefe`vre synd- rome. J Clin Periodontol 1983;10:618-35.
  • Stalder JF, Gellard P, Vergracht A, Bignon JD, Dreno B & Litoux P. Abnormalities in the expression of polymorphonuclear adhesion glyco- proteins after stimulation in two patients with Papillon- Lefe`vre syndrome. Card Grenoble Proceedings. J Invest Dermatol 1988;92: 146-51.
  • Umeda M, Zhang VJ, Kosaki T & Ishikawa I. Clinical, bacteriological and immunological exa- mination and treatment of two Papillon-Lefe`vre syndrome patients. J Oral Pathol 1990;57: 430-40.
  • Van Dyke TE, Taubman MA, Ebersole JL, Haffajee AD, Socransky SS, Smith DJ & Genco RJ. The Papillon- Lefe`vre syndrome: Neutrophil dys- function with severe periodontal disease. Clin Immunol Immunopathol 1984;31:419-29.
  • 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 synd- rome. J Periodontol Res 1982;17:563-8.
  • Preus HR & Morland B. In vitro studies of monocyte function in two siblings with Papillon- Lefe`vre syndrome. Scand J Dent Res 1987;95:59- 64.
  • Celenligil H, Kansu E, Ruacan S & Eratalay, K. Papillon-Lefe`vre syndrome. Characterization of peripheral blood and gingival lymphocytes with monoclonal 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.
  • Jung J, Carranza FA & Newman MG. Scanning electron-microscopy of plaque in Papillon- Lefe`vre syndrome. J Periodontol 1981;52:442-46.
  • Newman M, Angel I, Karge H, Weiner M, Grinenko V & Schusterman L. Bacterial studies of the Papillon-Lefe` vre Syndrome. J Dent Res 1977;56:545-46.
  • Preus HR Treatment of rapidly destructive periodontitis in Papillon-Lefe`vre syndrome. Laboratory and clinical observations. J Clin Periodontol 1988;15:639-43.
  • Eronat N, Ucar F & Kilnic G. Papillon- Lefe`vre syndrome: treatment of 2 cases with a cli- nical and microbiological investigation. J Clin Periodontol 1993;17: 99-104.
  • Ishikawa I, Umeda M & Laosrisin N. Clinical, bacteriological and immunological exami- nations and treatment process of two Papillon- Lefe`vre syndrome patients. J Periodontol 1994;65:364-71.
  • Tinanoff N, Tempro P & Maderazo EG. Dental treatment of Papillon-Lefe`vre syndrome: 15- year follow-up. J Clin Periodontol 1995;22:62-86.
  • De Vree H, Steenackers K, De Boever JA. Periodontal treatment of rapid progressive periodon- titis in 2 siblings with papillon-lefevre syndrome: 15- year follow up. J Clin Periodontol 2000;27:354-60.
  • Velazco CH, Coelho C, Salzar F, Contreras A, Slots J & Pacheco JJ. Microbiological features of Papillon–Lefe`vre syndrome periodontitis. J Clin Periodontol 1999;26:622-27.
  • Clerehugh V, Drucker DB, Seymour GJ & Bird PS. Microbiological and serological investiga- tion of oral lesions in Papillon–Lefe`vre syndrome. J Clin Pathol 1996;49:255-7.
  • Baer PN, McDonald RE. Suggested mode of periodontal therapy for patients with Papillon- Lefevre syndrome. Periodontol Case Rep 1981;1:10- 4.
  • Tinanoff N, Tanzer J, Kornman KS & Maderazo EG. Treatment of the periodontal compo- nent of Papillon-Lefe`vre syndrome. J Clin Periodontol 1986;13: 6-10.
  • Lu HKJ, Lin CT & Kwan HW. Treatment of a patient with Papillon- Lefe`vre syndrome: a case report. J Periodontol 1987;56:789-93.
  • Tinanoff N, Tanzer JM, Kornman KS & Maderazo EG. Treatment of the periodontal compo- nent of Papillon-Lefe`vre syndrome: A case report. J Clin Periodontol 1990;61:373-7.
  • Rüdiger S, Petersilka G, Flemmig TF. Combined systemic and local antimicrobial therapy of periodontal disease in Papillon-Lefevre syndrome. A report of 4 cases. J Clin Periodontol 1999;26:847- 54.
  • Lundgren T Crossner CG, Twetman S, Ulbro C. Systemic retinoid medication and periodon- tal health in patients with Papillon-Lefevre syn- drome. J Clin Periodontol 1996; 23:176-9.
  • Kressin S, Herforth A, Preis S, Wahn V & Lenard HG. Papillon-Lefe`vre syndrome-successful treatment with a combination of retinoid and concur- rent systematic periodontal therapy: Case reports. Quintessence Int 1995;26:795- 803.
  • Gelmetti C, Nazzaro V, Cerri D, Fracasso L. Long-term preservation of permanent teeth in a patient with Papillon-Lefevre syndrome treated with etretinate. Pediatr Dermatol 1989;6:222-5.
  • Gorlin RJ, Sedano H, Anderson VE, The syndrome of palmar-plantar hyperkeratosis and pre- mature periodontal destruction of the teeth. J Pediatr 1964;65:895-908.
There are 44 citations in total.

Details

Primary Language Turkish
Journal Section Research Article
Authors

Onur Özçelik This is me

M. Cenk Haytac This is me

Publication Date October 1, 2006
Published in Issue Year 2006 Volume: 33 Issue: 2 - Volume: 33 Issue: 2

Cite

Vancouver Özçelik O, Haytac MC. PAPILLON-LEFEVRE SENDROMU İLE İLİŞKİLİ PERİODONTİTİS OLGUSU: 1 YILLIK TAKİP. EADS. 2006;33(2):127-33.