Open AccessPapillon-Lefevre syndrome: Two case reports
Author(s) -
Jigna S Shah,
S.P. Goel
Publication year - 2007
Publication title -
indian journal of dental research/indian journal of dental research
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.277
H-Index - 43
eISSN - 1998-3603
pISSN - 0970-9290
DOI - 10.4103/0970-9290.35834
Subject(s) - medicine , dermatology , deciduous teeth , palmoplantar keratoderma , aggressive periodontitis , permanent teeth , periodontitis , dentistry , pediatrics , hyperkeratosis
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.