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Journals / Journal of Clinical and Experimental Investigations / 2026 / Cilt: 2 Sayı: 2

Three cases with papillon-lefevre syndrome

Papillon-Lefevre sendromlu üç olgu

Derya Uçmak
Yavuz Yeşilova
Mehmet Nurullah Er
Tuba Dal
Pages
222–224
Publication Date
2011-06-01
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Here we present a Papillon- Lefevre Syndrome case, which is rarely seen, with a review of the literature.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Papillon-Lefevre Syndrome is an extremely rare genetic disorder that typically effects infants of approximately one to 5 years of age.
📊
Key Findings & Data:In the general population, the disorder occurs in approximately one to 4 individuals per 1.000.000.
💡
Conclusion & Impact:Here we present a Papillon- Lefevre Syndrome case, which is rarely seen, with a review of the literature.
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Juvenile periodontitis · Papillon-Lefevre Sendromu · Palmoplantar hiperkeratoz

Abstract

Papillon-Lefevre Syndrome is an extremely rare genetic disorder that typically effects infants of approximately one to 5 years of age. Papillon-Lefevre Syndrome is characterized by the development of palmar-plantar hyperkeratosis and early loss of the primary (deciduous) and permanent teeth due to rapidly progressive periodontopathy. The primary (deciduous) teeth frequently become loose and fall out by about five years of age. In the general population, the disorder occurs in approximately one to 4 individuals per 1.000.000. Here we present a Papillon- Lefevre Syndrome case, which is rarely seen, with a review of the literature.

Özet

Papillon-Lefevre Sendromu, tipik olarak 1-5 yaş civarında ortaya çıkan son derece nadir genetik bir bozukluktur. Papillon-Lefevre Sendromu, palmoplantar hiperkeratoz ile başlayan ve hızla gelişen periodontopatiye bağlı olarak süt dişleri ve kalıcı dişlerin erken kaybıyla karakterizedir. Sütdişleri serbest hale gelir ve bunlar 5 yaş civarında kaybedilir. Genel populasyonda bozukluğun görülme sıklığı, yaklaşık milyonda 1-4 civarındadır. Nadir görülmesi nedeniyle Papillon-Lefevre Sendromlu üç olguyu ilgili literatür bilgileri ile birlikte sunuyoruz.

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Cite This Article

Uçmak, D., Yeşilova, Y., Er, M. N., Dal, T. (2011). Three cases with papillon-lefevre syndrome. Journal of Clinical and Experimental Investigations, Cilt: 2 Sayı: 2, 222–224. https://doi.org/10.5799/ahinjs.01.2011.02.0244

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References (12)

  1. Nazarro V, Blanchet-Bardon C, Mimoz C, Revuz J, Puis- sant A. Papillon-Lefevre syndrome. Arch Dermatol 1998;40(6):533-9.
  2. Pratchyapruit WO, Kullavavanijaya P. Papillon-Lefevre syn- drome: a case report. J Dermatol 2002;29(5):329-35.
  3. Gorlin RJ, Sedano H, Anderson VE. The syndrome of pal- marplantar hyperkeratosis and premature periodontal de- struction of the teeth. J Pediatr 1964;65(9):895-908.
  4. Wiebe CB, Hakkinen L, Putnins EE. Successful periodontal maintenance of a case with Papillon-Lefevre syndrome:12- year follow- up and reviewof literature. J Periodontol 2001;72(9):824-30.
  5. Hart TC, Hart PS, Bowden DW, et. al. Mutations of the cathe- psin C gene are responsible for Papillon-Lefevre syndrome. J Med Genet 1999;36(9):881-8.
  6. Toomes C, James J, Wood AJ, et al. Loss of function muta- tions in the cathepsin C gene result in periodontal disease and palmoplantar keratosis. Nat Genet 1999;23(5):421-4.
  7. Posteraro AF. Papillon-Lefevre syndrome. J Ala Dent Assoc 1992;76(1):16-25.
  8. 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.
  9. Pacheco JJ, Coelho C, Salazar F, Contreras A, Slots J, Ve- lazco CH. Treatment of Papillon-Lefevre syndrome perio- dontitis. J Clin Periodontol 2002;29(4):370-4.
  10. Rudiger S, Petersilka G, Flemming TF. Combined systemic and local antimicrobial therapy of periodontal disease in Papillon-Lefevre syndrome. A report of 4 cases. J Clin Periodontol 1999;26(9):847-54.
  11. Hart TC, Shapira L. Papillon-Lefevre syndrome. Periodon- tol 1994;6(2):88-100.
  12. Lundgren T, Renvert S, Papapanou PN, Dahlén G. Subgin- gival microbial profile of Papillon-Lefèvre patients assessed by DNA-probes. J Clin Periodontol 1998;25(8):624-9.