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CASE REPORT
Year : 2009  |  Volume : 21  |  Issue : 1  |  Page : 21-24

Papillon-Lefevre syndrome


Department of Oral Medicine, Diagnosis and Radiology, MS Ramaiah Dental College and Hospital, MSRIT Post, New BEL Road, Bangalore-560 054, Karnataka, India

Correspondence Address:
S Sujatha
Department of Oral Medicine, Diagnosis and Radiology, MS Ramaiah Dental College and Hospital, MSRIT Post, New BEL Road, Bangalore-560 054
India
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Source of Support: Dr. H.N. Shamao Rao, Principal and Professor, MS Ramaiah Dental College and Hospital, Bangalore, Conflict of Interest: None


DOI: 10.4103/0972-1363.57774

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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.


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