Papillon-Lefevre Syndrome: Report of two cases in the same family

Yıl 2012, Cilt: 15 Sayı: 2, 149 - 155, 19.04.2012

Arzu Yıldırım Bicer İlkay Peker Oguzhan Demirel

https://doi.org/10.7126/cdj.2012.803

Öz

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by the association of palmoplantar hyperkeratosis and premature loss of both deciduous and permanent  teeth. Two siblings (29-years-old female and 36-years-old male) with PLS who complained about early teeth loss, esthetic problems and difficulty during eating and speech refered to our clinic. After intraoral and radiologic examination we planned total prosthesis to first case’s upper and lower jaw and partial prosthesis to second patient’s upper and lower jaw. Patients’ financial problem affected the treatment planning. This case report presents prosthodontic rehabilitation of two patients with PLS in the same family.

Anahtar Kelimeler

Papillon Lefevre syndrome, Palmoplantar keratoderma, loss of dentition

Kaynakça

• Dhanrajani syndrome: clinical presentation and a brief review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod ;108(1):1-7.[CrossRef] Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmarplantar hyperkeratosis periodontal destruction of teeth. J Paediatr 1964;65:895-908. premature
• Hattab FN, Rawashdeh MA, Yassin OM, Al-Momani AS, Al- Ubosi MM. Papillon-Lefevre syndrome: a review of the literature and report of 4 cases. J CrossRef] Subramaniam P, Mathew S, Gupta KK. Papillon-Lefevre syndrome: A case report. J Indian Soc Pedod Prevent Dent 2008;26(4):171-174. CrossRef]
• Ullbro C, Twetman S. Dental treatment for patients with Papillon- Lefevre syndrome. Review article. Eur Arch Paediatr Dent 2007;8(Suppl ):4-11.
• Hancke E. The Papillon Lefevre syndrome: Keratosis palmar plantaris with periodontopathy: Report of a case and review of cases in the literature. Hum Genet 1979;51:1-35. Janjua
• Papillon-Lefevre syndrome: Case report and review of the literature. Dermatol Online J 2004;10:13-19.
• Kothiwale SV, Mathur S. Partial expression Syndrome. Indian J dent Res ;19:264-266.[CrossRef] Ullbro C, Crosner CG, Nederfos T, Thelstrup-Pederson K. Dermatologic and oral finding in a cohort of 47 patients with Papillon Lefevre syndrome. J Am Acad Dermatol ;48:45-51.[CrossRef] Pilger U, Hennies HC, Truschnegg A, Aberer E. Late-onset Papillon- Lefevre syndrome without alteration of cathepsin C gene. J Am Acad Dermatol 2003;49:240-243.
• Singla A, Sheikh S, Jindal SK, Brar R. Bridge between Dermatologist and Dentist. J Clin Exp Dent 2010;2:e43- [CrossRef] syndrome:
• Hart TC, Shapira L. Papillon-Lefevre syndrome: keratosis palmoplantaris. Periodontol 2000;1994:88-100.
• Bindayel NA, Ullbro C, Suri L, Al- Farra E. Cephalometric findings in patients syndrome. Am J Orthod Dentofacial Orthop CrossRef] Papillon-Lefevre ;134(1):138-144. Angel TA, Hsu S, Kornbleuth J, Kramer syndrome, a case report of four affected siblings. J Am Acad Dermatol 2002;46(2 Suppl. Case Reports):8-10.[CrossRef]
• Jain V, Gupta R, Parkash H. Prosthodontic Papillon-Lefevre syndrome: a case report. J Indian Soc Pedod Prevent Dent 2005;23(2):96-98. in CrossRef]