1887
Volume 2008, Issue 2
  • ISSN: 0253-8253
  • E-ISSN: 2227-0426

Abstract

Papillon-Lefevre syndrome (PLS), first described in 1924, is a rare autosomal recessive disorder characterized by early onset palmoplanter hyperkeratosis and severe generalized destructive periodontitis leading to premature loss of both primary and permanent dentitions. PLS usually manifest itself between the ages of six months to four years, coinciding with the eruption of primary teeth. The proband becomes completely edentulous by the age of 15.

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2008-12-01
2019-10-14
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References

  1. 1. Papillon   MM., , Lefevre   P. . Deux cas de keratoderma palmaire et plantaire symmetrique familiale (maladie de Meleda) chez le frere et la soeur: coexistence dans les deux cas d>alterations dentaires graves. . Bull Soc Fr Dermatol Syph . 1924; ;31: : 82– 87 .
    [Google Scholar]
  2. 2. Hart   T., , Shapira   L. . Papillon-Lefevre syndrome. . Periodontol 2000 . 1994; ;6: : 88– 100 .
    [Google Scholar]
  3. 3. Hattab   FN., , Rawashdeh   MA., , Yassin   OM. . Papillon-Lefevre syndrome: A review of the literature and report of 4 cases. . J Periodontol . 1995; ;66: : 413– 420 .
    [Google Scholar]
  4. 4. Gorlin   RJ., , Sedano   H., , Anderson   VE. . The syndrome of palmarthe teeth. . J Pediatr . 1964; ;65: : 895– 908 .
    [Google Scholar]
  5. 5. Haneke   E. . The Papillon-Lefevre syndrome: Keratosis palmoplantaris with periodontopathy: Report of a case and review of the cases in the literature. . Hum Genet . 1979; ;51: : 1– 35 .
    [Google Scholar]
  6. 6. Haneke   E., , Hornstein   OP., , Lex   C. . Increased susceptibility to infections in Papillon-Lefevre syndrome. . Dermatologica . 1975; ;150: : 283– 286 .
    [Google Scholar]
  7. 7. Almuneef   M., , Al Khenaizan   S., , Al Ajaji   S. . Pyogenic liver abscess and Papillon-Lefevre syndrome: not a rare association. . Pediatrics . 2003; ;111: : 85– 88 .
    [Google Scholar]
  8. 8. Baghdady   VS. . Papillon-Lefevre syndrome: report of four cases. . ASDC J Dent Child . 1982; ;49: : 147– 150 .
    [Google Scholar]
  9. 9. Hattab   FN., , Amin   WM. . Papillon-Lefevre syndrome with albinism: A review of the literature and report of two brothers. . Oral Surg Oral Med Oral Pathol Oral Radiol Endod . 2005; ;100: : 709– 716 .
    [Google Scholar]
  10. 10. Khoury   SA., , Massad   DD. . Consanguineous marriage in Jordan. . Am J Med Genet . 1992; ;43: : 206– 210 .
    [Google Scholar]
  11. 11. Altalabani   J., , Shubbar   AI., , Mustafa   KE. . Major congenital malformations in the United Arab Emirates (UAE): Need for genetic counselling. . Ann Hum Genet . 1998; ;62: : 411– 418 .
    [Google Scholar]
  12. 12. Randovanovic   Z., , Shah   N., , Behbehani   J. . Prevalence and social correlations of cosanguinity in Kuwait. . Ann Saudi Med . 1999; ;19: : 206– 210 .
    [Google Scholar]
  13. 13. Laass   MW., , Hennis   HC., , Preis   S. . Localization of a gene for Papillon-Lefevre syndrome to chromosome Ilql4-q21 by homozygosity mapping. . Hum Genet . 1997; ;101: : 376– 382 .
    [Google Scholar]
  14. 14. Toomes   C., , James   J., , Wood   AJ. . Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplanter keratosis. . Nat Genet . 1999; ;23: : 421– 424 .
    [Google Scholar]
  15. 15. Cury   VF., , Costa   JE., , Gomez   RS. . A novel mutation of the cathepsin C in Papillon-Lefevre syndrome. . J Periodontol . 2002; ;73: : 307– 312 .
    [Google Scholar]
  16. 16. Hart   TC., , Hart   PS., , Michalec   MD. . Haim-Munk syndrome and Papillon-Lefevre syndrome are allelic mutations in cathepsin C. . J Med Genet . 2000; ;37: : 88– 94 .
    [Google Scholar]
  17. 17. Hart   TC., , Hart   PS., , Michalec   MD. . Localization of a gene for prepubertal periodontitis to chromosome llql4 and identification of cathepsin C gene mutation. . J Med Genet . 2000; ;37: : 95– 101 .
    [Google Scholar]
  18. 18. Hewin   C., , McCormick   D., , Linden   G. . The role of cathepsin C in the Papillon-Lefevre syndrome, prepubertal periodontitis, and aggressive periodontitis. . Hum Mutat . 2004; ;23: : 222– 228 .
    [Google Scholar]
  19. 19. Drucker   DB., , Marshall., , Bird   PS. . Aetiology of Papillon-Lefevre syndrome. . Oral Dent Bacteriol Infect . 2001; ; : 151– 158 .
    [Google Scholar]
  20. 20. Pilger   U., , Hennies   HC., , Truschnegg   A. . Late-onset Papillon-Lefevre syndrome without alteration of the cathepsin C gene. . J Am Acad Dermatol . 2003; ;49: : S240– S243 .
    [Google Scholar]
  21. 21. Hewin   C., , Wu   CL., , Hattab   FN. . Co-inheritance of two rare genodermatoses (Papillon-Lefevre syndrome and oculocutaneous albinism type 1) in two families: a genetic study. . Br J Dermatol . 2004; ;151: : 1261– 1265 .
    [Google Scholar]
  22. 22. Kellum   RE. . Papillon-Lefevre syndrome in four siblings treated with etretinate: A nine-year evaluation. . Int J Dermatol . 1989; ;28: : 605– 608 .
    [Google Scholar]
  23. 23. Bravo-Piris   J., , Aprarico   M., , Moran   M. . Papillon-Lefevre syndrome: Report of a case treated with oral retinoid RO 10-9359. . Dermatologica . 1983; ;166: : 97– 103 .
    [Google Scholar]
  24. 24. Nazzaro   V., , Blanchet-Bardon   C. . Papillon-Lefevre syndrome: Ultrastructural study and successful treatment with acitretin. . Arch Dermatol . 1988; ;124: : 533– 539 .
    [Google Scholar]
  25. 25. Gelmetti   C., , Nazzaro   V., , Cerri   D. . Long-term preservation of permanent teeth in a patient with Papillon-Lefevre syndrome treated with etretinate. . Pediatr Dermatol . 1989; ;6: : 222– 225 .
    [Google Scholar]
  26. 26. Tinanoff   N., , Tanzer   JM., , Kornman   KS. . Treatment of the periodontal component of Papillon-Lefevre syndrome. . J Clin Periodontol . 1986; ;13: : 6– 10 .
    [Google Scholar]
  27. 27. Preus   HR. . Treatment of rapidly destructive periodontitis in Papillon-Lefevre syndrome: laboratory and clinical observations. . J Clin Periodontol . 1988; ;15: : 639– 643 .
    [Google Scholar]
  28. 28. Glenwright   HD., , Rock   WP. . Papillon-Lefevre syndrome: A case discussion of aetiology and a case report. . Br Dent J; . 1990; ;168: : 27– 29 .
    [Google Scholar]
  29. 29. De Vree   H., , Steenackers   K., , De Boever   JA. . Periodontal treatment of rapid progressive periodontitis in 2 siblings with Papillon-Lefevre syndrome: 15-year follow-up. . J Clin Periodontol . 2000; ;27: : 354– 360 .
    [Google Scholar]
  30. 30. Eronat   N., , Ucar   F., , Kilinc   G. . Papillon-Lefevre syndrome: Treatment of two cases with a clinical microbiological and histopathological investigation. . J Clin Pediatar Dent . 1993; ;17: : 99– 104 .
    [Google Scholar]
  31. 31. Pacheco   JJ., , Coelho   C., , Salazar   F. . Treatment of Papillon-Lefevre syndrome periodontitis. . J Clin Periodontol . 2002; ;29: : 370– 374 .
    [Google Scholar]
  32. 32. Ullbro   C., , Crossner   UC., , Lundgren   C-G. . Osseointegrated implants in a patient with Papillon-Lefevre syndrome: A 41/2-year followup. . J Clin Periodontol . 2000; ;27: : 951– 954 .
    [Google Scholar]
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