Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma

Papillon–Lefevre syndrome is an autosomal recessive disorder distinguished mainly by a triad of palmar-plantar hyperkeratosis, aggressive periodontitis involving both deciduous and permanent dentition and intracranial calcifications. Patients also exhibit increased susceptibility to bacterial infections. Most of the cases are reported on account of homozygous mutations affecting cathepsin C (CTSC) gene, as both the alleles of cathepsin C gene are mutated. Periodontal disease can, however, develop either as a principal manifestation or be fostered by the presence of local factors. This article presents such a rare case report along with dermatological manifestations and its clinical significance from dental perspective. This will aid in a proper multidisciplinary treatment approach.