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Category: Volume 07 Issue 04 April 2019
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Title: Papillon Lefèvre syndrome – Rare or Underdiagnosed?

Authors: Dr Amit Chauhan, Dr Ghanshyam Verma, Dr Gita Ram Tegta

 DOI: https://dx.doi.org/10.18535/jmscr/v7i4.139

Abstract

Papillon Lefèvre syndrome represents an autosomal recessive condition being characterised by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis leading to premature loss of both primary and permanent dentitions. Incidence varies from 1 to 4 persons per million. The available literature emphasises the need for a collaborative effort on the part of dermatologist as well as dentist for diagnosing this syndrome at an early age thus to prevent further complications. We report a case of Papillon Lefèvre syndrome along with treatment and follow up of the patient.

Keywords: Papillon-Lefèvre syndrome, Hyperkeratosis, Periodontitis.

References

  1. Muppa R, Prameela B, Duddu M, Dandempally A. Papillon-lefevre syndrome: A combined approach from the dermatologist and dentist — A clinical presentation. Indian J Dermatol 2011; 56:740-3.
  2. Hart TC, Shapira L. Papillon-Lefèvre syndrome. Periodontol 2000 1994; 6:88-100.
  3. Dhanrajani PJ. Papillon-Lefevre syndrome: Clinical presentation and a brief review. Oral Surg Oral Med Oral Pathol Oral RadiolEndod 2009;108: e1-7.
  4. Yang Y, Bai X, Liu H, Li L, Cao C, Ge L. Novel mutations of cathepsin C gene in two Chinese patients with Papillon-Lefèvre syndrome. J Dent Res 2007; 86:735-8.
  5. Kansky AA, Potocnik M, Kansky A. Papillon-Lefèvre syndrome. Acta Dermatovenerological A 1997; 6:60-5.
  6. KordValeshabad A, Mazidi A. Papillon-lefèvre syndrome: A series of six cases in the same family. ISRN Dermatol 2012; 2012:139104.
  7. Thakker N. Genetic analysis of Papillon –Lefevre syndrome. Oral Dis. 2000; 6:263.
  8. Angel TA, Hsu S, Kornbleuth SI, Kornbleuth J, Kramer EM. Papillon–Lefevre syndrome: A case report of four affected siblings. J Am Acad Dermatol 2002;46: S8–10.
  9. Allende LM, Moreno A, de Unamuno P. A genetic study of cathepsin C gene in two families with Papillon-Lefèvre syndrome. Mol Genet Metab. 2003; 79:146–8.
  10. Umeda M, Zhang VJ, Koseki T, Ishikawa I. Clinical, bacteriological and immunological examination and treatment of two Papillon-Lefèvre syndrome patients. Kokubyo Gakkai Zasshi. 1990;57(3):430–440.
  11. Nagaveni NB, Suma R, Shashikiran ND, Subba Reddy VV. The Papillon-Lefevre syndrome: Report of two cases in the same family. J Indian Soc PedodPrev Dent. 2008; 26:78–81.
  12. Ullbro C, Brown A, Twetman S. Preventive periodontal regimen in Papillon-Lefèvre syndrome. Pediatr Dent 2005; 27:226–32.
  13. Singh R, Nor M, Ghazali W. Atypical Papiilon-Lefèvre syndrome: keratosis palmoplantaris with periodontopathy. Int J Dermatol. 1993; 32:450–52.
  14. BerUllbro 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; 48:345-51.

Singla A, Sheikh S, Jindal SK, Brar R. Papillon Lefevre syndrome: Bridge between dermatologist and dentist. J Clin Exp Dent 2010;2:e43-6.

Corresponding Author

Dr Ghanshyam Verma

Associate Professor Dept of Dermatology, Venereology Indira Gandhi Medical College Shimla HP. India

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