Prosthetic Rehabilitation in Partially Edentulous Children with Papillon-Lefèvre Syndrome: Two Case Reports

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disease characterized by palmoplantar keratosis and early-onset severe periodontitis. This disease affects both primary and permanent teeth, causing premature tooth loss. Various etiological factors are associated with PLS, including genetic mutations, immunological changes, and bacterial effects. The genetic defect associated with PLS has been shown to result from a mutation in the Cathepsin C gene, located on the long arm of chromosome 11 (11q14). Removable prostheses allow changes during the growth and development of the jaw bones in PLS patients, providing the function and aesthetics that are lost until the jaw development is complete. These case reports include two siblings (7-year-old boy and 11-year-old girl) with PLS and their prosthetic rehabilitation with partial removable prostheses with a multidisciplinary approach. Routine check-ups of the patients every three months have been continuing for approximately 6 months.