Abstract

Papillon-Lefèvre Syndrome (PLS) is a rare genetic disorder, characterized by Palmoplantar keratosis, aggressive periodontitis and premature edentulous primary and permanent dentition at a very young age. Additional symptoms and findings associated with PLS may include frequent pyogenic skin infections, abnormalities of the nails, and excessive perspiration. Papillon-Lefèvre syndrome is inherited in an autosomal recessive pattern. The various etiological and pathogenic factors are associated with the syndrome: immunologic alterations, genetic mutation and the role of the pathogenic microorganism. Dentists, especially pediatric dentist is very important specialist to diagnosis and management of PLS, there were the periodontal destruction at early childhood. We introduce the clinical presentation and management options of 5 years old boy with PLS in Mongolia.