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.
Keyword :
Cathepsin C Gene, Genodermal Disorder, Palmoplantar Hyperkeratosis, Periodontitis