Papillon-Lefevre syndrome: Report of two cases in the same family.
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2008-04-08
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Abstract
Papillon-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.
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Nagaveni 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): 78-81