| CASE REPORT |
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| Year : 2008 | Volume
: 26
| Issue : 2 | Page : 78-81 |
Papillon-Lefevre syndrome: Report of two cases in the same family
NB Nagaveni, R Suma, ND Shashikiran, VV Subba Reddy
Department of Pedodontics, College of Dental Sciences, Davangere - 577 004, Karnataka, India
Correspondence Address:
N B Nagaveni
Department of Pedodontics, College of Dental Sciences, Davangere - 577 004, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-4388.41622
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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