Papillon-Lefèvre Sendromlu Kısmen Dişsiz Çocuklarda Protetik Rehabilitasyon: İki Olgu Sunumu

Year 2025, Volume: 12 Issue: 2, 321 - 325, 22.08.2025

Fatma Güneş , Merve Öge , Mustafa Kocacıklı , Haluk Bodur

https://doi.org/10.15311/selcukdentj.1542742

Abstract

Papillon-Lefèvre sendromu (PLS), palmoplantar keratoz ve erken başlangıçlı şiddetli periodontitis ile karakterize nadir bir otozomal resesif hastalıktır. PLS hem süt hem de kalıcı dişleri etkileyerek dişlerin erken kaybına neden olur. Genetik mutasyonlar, immünolojik değişiklikler ve bakteriyel etkiler dahil olmak üzere çeşitli etiyolojik faktörler PLS ile ilişkilidir. PLS ile ilişkili genetik defektin, kromozom 11'in uzun kolunda (11q14) lokalize olan Katepsin C genindeki bir mutasyondan kaynaklandığı gösterilmiştir. Hareketli protezler, PLS hastalarında çene kemiklerinin büyümesi ve gelişimi sırasında değişikliklere izin vererek, çene gelişimi tamamlanana kadar kaybedilen fonksiyon ve estetiği sağlar. Bu vaka raporları, multidisiplener bir yaklaşımla PLS'li iki kardeşi (7 yaşında erkek ve 11 yaşında kız) ve kısmi hareketli protezlerle protetik rehabilitasyonlarını içermektedir. Hastaların her üç ayda bir rutin kontrolleri yaklaşık 6 aydır devam etmektedir.

Keywords

hareketli parsiyel protez , Papillon-Lefèvre Sendrom , protetik rehabilitasyon

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

Abstract

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.

Keywords

Papillon-Lefèvre syndrome , Prosthetic Rehabilitation , Removable partial denture

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