Papillon-lefèvre syndrome: an unusual case report

Gökhan Özkan, Aydan Kanlı, Saniye Eren Halıcı

Abstract


Papillon-Lefèvre syndrome (PLS) is a rare autosomal disorder characterized by palmoplantar hyperkeratosis and periodontal problems leading to premature loss of both primary and permanent dentitions. It is caused by mutation of cathepsin C gene generating deficiency in host immune response to microorganisms. Up to date, no sexual or racial predominance has been detected. This paper report the diagnosis and rehabilitation procedures for a 14-year-old male patient with PLS .


Keywords


Cathepsin C; hyperkeratosis; Papillon-Lefèvre syndrome; prosthetic rehabilitation

References


Papillon MM, Lefévre P. 2 cases of symmetrically, familiarly palmar and plantar hyperkeratosis (Meleda disease) within brother and sister combined with severe dental alterations in both cases. Soc Franc Dermat Syph. 1924;31:82-4.

Veerabahu BG, Chandrasekaran S, Alam MN, Krishnan M. Papillon–Lefévre syndrome. Journal of oral and maxillofacial pathology: JOMFP. 2011;15(3):352-7.

Cawson RA, Odell EW. Cawson’s essentials of oral pathology and oral medicine. Philadelphia: Churchill Livingstone; 2008.

Sloan P, Soames J, Murray J, Jenkins W. Histopathological and ultrastructural findings in a case of Papillon-Lefevre syndrome. Journal of periodontology. 1984;55(8):482-5.

Gorlin R, Sedano H, Anderson V. The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth: a clinical and genetic analysis of the Papillon-Lefèvre syndrome. The Journal of pediatrics. 1964;65(6):895-908.

Haneke E. The Papillon-Lefèvre syndrome: keratosis palmoplantaris with periodontopathy. Human genetics. 1979;51(1):1-35.

Dalgic B, Bukulmez A, Sari S. Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon–Lefevre syndrome. European journal of pediatrics. 2011;170(6):803-5.

Hart TC, Shapira L. Papillon‐Lefèvre syndrome. Periodontology 2000. 1994;6(1):88-100.

Farkas K, Paschali E, Papp F, Vályi P, Széll M, Kemény L, et al. A novel seven-base deletion of the CTSC gene identified in a Hungarian family with Papillon-Lefèvre syndrome. Archives of dermatological research. 2013;305(5):453-5.

Newman M, Angel I, Karge H, Weiner M, Grinenko V, Schusterman L. Bacterial studies of the Papillon-Lefevre syndrome. Journal of dental research. 1977;56(5):545.

Muppa R, Prameela B, Duddu M, Dandempally A. Papillon-lefevre syndrome: A combined approach from the dermatologist and dentist-A clinical presentation. Indian journal of dermatology. 2011;56(6):740-3.

Nickles K, Schacher B, Schuster G, Valesky E, Eickholz P. Evaluation of two siblings with Papillon-Lefèvre syndrome 5 years after treatment of periodontitis in primary and mixed dentition. Journal of periodontology. 2011;82(11):1536-47.

Wara-aswapati N, Lertsirivorakul J, Nagasawa T, Kawashima Y, Ishikawa I. Papillon-Lefevre syndrome: serum immunoglobulin G (IgG) subclass antibody response to periodontopathic bacteria. A case report. Journal of periodontology. 2001;72(12):1747-54.

Pacheco J, Coelho C, Salazar F, Contreras A, Slots J, Velazco C. Treatment of Papillon‐Lefèvre syndrome periodontitis. Journal of clinical periodontology. 2002;29(4):370-4.

Kressin S, Herforth A, Preis S, Wahn V, Lenard H. Papillon-Lefèvre syndrome-Successful treatment with a combination of retinoid and concurrent systematic periodontal therapy: Case reports. Quintessence International. 1995;26(11):795-803.


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