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Papillon?Lefevre Syndrome: A Case Report with Review of Literature

Journal: Indian Journal of Mednodent and Allied Sciences (Vol.2, No. 1)

Publication Date:

Authors : ; ;

Page : 105-108

Keywords : Autosomal recessive disorder; Palmoplantar keratosis; Periodontitis; Premature teeth loss; Keratoderma;

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Abstract

Papillon?Lefevre Syndrome(PLS) is a very rare syndrome of autosomal recessive inheritance characterised 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. We report a case of 17-year- old female patient withPLS.

Last modified: 2015-06-19 18:11:21