Papillon-Lefevre syndrome: two case reports.
Papillon-Lefevre syndrome is a rare autosomal recessive disorder in which there is palmoplantar keratinization and premature loss of both deciduous and permanent teeth. The palmoplantar keratoderma typically has its onset between the ages of 1 and 4 years and severe periodontitis starts at the age of 3 or 4 years. An early diagnosis of the syndrome can help preserve the teeth by early institution of treatment, using a multidisciplinary approach. We present two cases of the syndrome having all of the characteristic features.
Shah
Jigna
J
Department of Oral Medicine and Radiology, G.D.C.H, Ahmedabad, Gujarat - 380 016, India. sathya_opth@yahoo.com
Goel
Shweta
S
D000293:Adolescent; D016301:Alveolar Bone Loss / Q000175:diagnosis; D005260:Female; D005891:Gingivitis / Q000175:diagnosis; D006801:Humans; D008297:Male; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000235:genetics; D010518:Periodontitis / Q000175:diagnosis; D014080:Tooth Exfoliation / Q000175:diagnosis