Open AccessPapillon-Lefevre syndrome
Author(s) -
S. Sujatha,
Namita Raghav
Publication year - 2009
Publication title -
journal of indian academy of oral medicine and radiology
Language(s) - English
Resource type - Journals
eISSN - 0975-1572
pISSN - 0972-1363
DOI - 10.4103/0972-1363.57774
Subject(s) - medicine , periodontitis , dermatology , keratoderma , hyperkeratosis , pediatrics , dentistry
Papillon-Lefevre syndrome (PLS) 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. The palmar-plantar 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. The exact pathogenesis of these clinical events remains mainly speculative. An early diagnosis of the syndrome can help preserve the teeth by early institution of treatment, using a multidisciplinary approach. This paper describes a case of PLS with classic clinical features and briefly reviews the relevant literature