Title: Papillon-Lefevre Syndrome with Aggressive Periodontitis - A Case Report with Review of Literature
Authors: Dr Suryakanth Malgikar*, Dr Madanika P, Dr Venkatesh Suneel B
DOI: http://dx.doi.org/10.18535/jmscr/v3i10.09
Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefevre in 1924. Papillon-Lefevre Syndrome (PLS) is described as the association of palmar-plantar hyperkeratosis with precocious periodontal disease which results in exfoliation of primary and permanent dentitions. The majority of the teeth are lost by the age of 14 to 15 years. This case report describes the periodontal findings and dermatological findings with review of literature. Keywords:Papillon-Lefevre syndrome, Palmoplantar hyperkeratosis, Keratodermas, Rapidly progressing periodontitis. 1. Papillon MM, LeFevre P. Deux cas de keratodemie palmaire et plantaire symetrique familiale (Malide de Meleda) chez le frere et la soeur. Coexistence dens le deux d'alterations dentaires graves. Bull Soc Fran Dermatol Syphil 1924;31: 82-84. 2. Erhan Firatli, Binnur Tüzün, and Ahmet Efeo lu. Papillon-Lefevre Syndrome. Analysis of Neutrophil Chemotaxis. J Periodontol 1996; 67;617-620. 3. Hart PS, Zhang Y, Firatli E, et al. Identification of cathepsin C mutations in ethnically diverse papillon-Lefevre syndrome patients. J Med Genet 2000; 37: 927-932. 4. Haneke E. The Papillon–Lefevre syndrome: keratosis palmoplantaris with periodontopathy. Human Genetics 1979; 51: 1–35. 5. Kaustubh Suresh Thakare, M. L. Bhongade, Pretti Charde, Shweta Kale, Priyanka Jaiswal, B. K. Somnath, and Sunil Pendor. Genetic Mapping in Papillon-Lefevre Syndrome: A Report of Two Cases. Case Reports in Dentistry. Volume 2013. 6. Jasim M. Albandar, Razan Khattab, Fawza Monem, Sara M. Barbuto, and Bruce J. Paster. The Subgingival Microbiota of Papillon-Lefevre Syndrome. J Periodontol 2012; 83: 902-908.Abstract
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