Prosthodontic rehabilitation in Papillon-Lefevre syndrome: A case report

Objectives: : Papillon Lefevre Syndrome (PLS) is an autosomal recessive (AR) disorder affecting the skin and intra oral soft tissues resulting in palmo plantar hyperkeratosis with premature periodontal problems leading to early tooth loss and associated functional and psychological disturbances. Functional, esthetics, developmental and psychological problems may arise in the PapillonLefevre Syndrome during childhood. The disorder occurs in approximately 1 to 4 individuals per 1.000.000. The exact etiology is still unknown. Patients are often edentulous at an early age.
Methods: A 14-year-old female patient presented to the department of Prosthodontic, Faculty of Dentistry,Gaziantep University, Gaziantep, Turkey, with the chief complaint of multiple loss of teeth. Most of her natural dentition was missing. The remaining four natural teeth had periodontal lesions. She was presented with the chief complaint of loss of many permanent teeth and mobility of the remaining teeth. Most of the teeth were lost very early after their eruption due to swollen infected gums. There were no other associated problems except for the mild burning sensation of the palms and soles. A decision was made and remaining teeth were extracted in order to prevent aggressive bone loss. She was then managed using removable partial dentures with bilateral balanced occlusion with little retentive help from the most posterior molars.
Results: The patient is comfortable and functioning well with her new dentition. Prosthodontic rehabilitation provides a psychological boost up to the patient as well as to the parents by restoring not only the esthetic appearance but also the function. The PLS debilitates individuals socially, psychologically, and physically.
Conclusions: Thus, prosthetic replacement in such patients is an age specific, speciality treatment involving initial replacement with complete or partial dentures and future consideration for an implant-supported prosthesis