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 interv… Show more
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