Prosthodontic rehabilitation of Papillon Lefevre Syndrome

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. This report presents management of a 17 years old girl suffering from PLS. 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 complete dentures with bilateral balanced occlusion. The patient is comfortable and functioning well with her new dentition.