Case Report Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology

Kundoor Reddy, Kotya Maloth, Nayanala Anusha, Venkata Thummala, Moni Thakur, & Head, P Student
2016 unpublished
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.