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

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dc.contributor.authorNagaveni, N Ben_US
dc.contributor.authorSuma, Ren_US
dc.contributor.authorShashikiran, N Den_US
dc.contributor.authorSubba Reddy, V Ven_US
dc.date.accessioned2008-04-08en_US
dc.date.accessioned2009-06-02T09:15:02Z
dc.date.available2008-04-08en_US
dc.date.available2009-06-02T09:15:02Z
dc.date.issued2008-04-08en_US
dc.description.abstractPapillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of the cathepsin C gene. Two cases of Papillon-Lefevre syndrome in the same family, having all of the characteristic features, are presented. An 11-year-old girl and a 9-year-old boy presented with the complaints of loose teeth. Both expressed hyperkeratosis of palms, soles, and knees. Severe generalized periodontal destruction, with mobility of teeth, was evident on intraoral examination; orthopantomograph examination showed severe generalized loss of alveolar bone in both the patients.en_US
dc.description.affiliationDepartment of Pedodontics, College of Dental Sciences, Davangere - 577 004, Karnataka, India. nagavenishankar@indiatimes.com.en_US
dc.identifier.citationNagaveni NB, Suma R, Shashikiran ND, Subba Reddy VV. Papillon-Lefevre syndrome: Report of two cases in the same family. Journal of the Indian Society of Pedodontics and Preventive Dentistry. 2008 Apr-Jun; 26(2): 78-81en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/114761
dc.language.isoengen_US
dc.source.urihttps://medind.nic.in/jao/jaoaj.shtmlen_US
dc.titlePapillon-Lefevre syndrome: Report of two cases in the same family.en_US
dc.typeJournal Articleen_US
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