Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology
  • Year: 2016
  • Volume: 2
  • Issue: 4

Papillon Lefevre Syndrome: A case series with review of literature

  • Author:
  • Kundoor V.K. Reddy1, Kotya N. Maloth2,, Nayanala V. Anusha3, Venkata S.R Thummala4, Moni Thakur2
  • Total Page Count: 4
  • Page Number: 248 to 251

1Professor & Head, Dept. of Oral Medicine & Radiology, Mamata Dental College & Hospital, Khammam, Telangana

2Senior Lecturer, Dept. of Oral Medicine & Radiology, Mamata Dental College & Hospital, Khammam, Telangana

3PG Student, Dept. of Oral Medicine & Radiology, Mamata Dental College & Hospital, Khammam, Telangana

4Associate Professor, Dept. of Oral Medicine & Radiology, Mamata Dental College & Hospital, Khammam, Telangana

*Corresponding Author: Email: dr.kotyanaik.maloth@gmail.com

Online published on 20 June, 2017.

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