Papillon–Lefevre Syndrome: A Case Report with Review of Literature Raghoji S Swetha1,*, Ravishankar T Shilpa2,** 1Asst. Professor, Dept of Oral & Maxillofacial Pathology, HKDET'S Dental College & Hospital, Humnabad-585 330, Karnataka 2Asst. Professor, Dept of Oral Medicine & Radiology, Navodaya Dental College & Hospital, Raichur-584 101, Karnataka Correspondence Author Email: *drswetha.op@gmail.com
**dr_shilpa55@yahoo.com
Online published on 15 May, 2014. 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 with PLS. Top Keywords Autosomal recessive disorder, Palmoplantar keratosis, Periodontitis, Premature teeth loss, Keratoderma. Top |