Papillon-Lefevre Syndrome

Abstract

ABSTRACT Papillon-Lefevre syndrome (PLS) is a genetically inherited disorder characterized by palmoplantar hyperkeratosis and severe early onset periodontitis. Oral symptoms begin to appear when primary teeth are in the process of erupting. A defect in the cathepsin C gene causes a dysfunction of the immune response occurring 1 to 3 times in 1 million births. Varying degrees of severity have been reported, ranging from complete edentulism at a young age to complete retention of teeth with a good prognosis. Early intervention and proper treatment has been shown to improve prognosis. The pathogenesis of PLS is not clearly defined and has been associated with microbiologic, immunologic and genetic factors. Treatment includes conventional periodontal therapies, strict recall and maintenance, as well as antibiotics, metronidazole and chlorhexidine prescriptions as needed. Concurrently, patients are typically under the care of a dermatologist and taking retinoid medications to control their skin condition. This case report discusses the ongoing care of a patient who first presented to NYU College of Dentistry in the pediatric clinic at age 4. It is suggested that periodontal treatment initiated early in life may significantly change the prognosis for the better. How to cite this article Congiusta MA, Koo D, Penugonda B, Goren AD, Kamer AR. Papillon-Lefevre Syndrome. Int J Experiment Dent Sci 2013;2(1):66-69.