This work is licensed under Creative Commons Attribution–NonCommercial International License
(CC BY-NC 4.0).
Background: Papillon-Lefevre 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.
Case description: A 3.6-year-old male patient reported to the central dental hospital, with a complaint of tongue
ulcer and exposed root of deciduous teeth. Intraoral examination of the patient had shown the absence of central
incisors in maxilla and in the mandible presence of first primary premolars and right primary canine. The anterior
third of the tongue was a traumatic ulcer in the maxillary left canine and lateral incisor.
Practical implications: We have followed up patient with PLS and the traumatic tongue ulcer of patient with
immunodeficiency was recovered. Dentists are very important specialists for the diagnosis and management of PLS.