Papillon Lefevre Syndrome: A case series with review of literature

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Case Report

Author Details : Kundoor V.K. Reddy, Kotya N. Maloth, Nayanala V. Anusha, Venkata S.R Thummala, Moni Thakur

Volume : 2, Issue : 4, Year : 2016

Article Page : 248-251

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Abstract

Papillon Lefevre syndrome (PLS) is a rare autosomal recessive inherited genodermal disorder, caused by cathepsin C gene mutation leading to the deficiency of cathepsin C enzymatic activity and consanguinity of parents is evident in about one third of cases. The disorder is characterized by palmoplantar hyperkeratosis and periodontitis that results in premature loss of deciduous and permanent teeth. Here we report a case series of PLS with typical clinical and radiographic features.

Keywords: Cathepsin C Gene, Genodermal Disorder, Palmoplantar Hyperkeratosis, Periodontitis

How to cite : Reddy K V, Maloth K N, Anusha N V, Thummala V S, Thakur M, Papillon Lefevre Syndrome: A case series with review of literature. J Oral Med Oral Surg Oral Pathol Oral Radiol 2016;2(4):248-251

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