Journal of Indian Society of Pedodontics and Preventive Dentistry
Journal of Indian Society of Pedodontics and Preventive Dentistry
                                                   Official journal of the Indian Society of Pedodontics and Preventive Dentistry                           
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

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.


How to cite this article:
Nagaveni N B, Suma R, Shashikiran N D, Subba Reddy V V. Papillon-Lefevre syndrome: Report of two cases in the same family.J Indian Soc Pedod Prev Dent 2008;26:78-81


How to cite this URL:
Nagaveni N B, Suma R, Shashikiran N D, Subba Reddy V V. Papillon-Lefevre syndrome: Report of two cases in the same family. J Indian Soc Pedod Prev Dent [serial online] 2008 [cited 2019 Dec 13 ];26:78-81
Available from: http://www.jisppd.com/article.asp?issn=0970-4388;year=2008;volume=26;issue=2;spage=78;epage=81;aulast=Nagaveni;type=0