Çege M. A., Çankal D. A., Binici Aygün E., Turhan Bal B.
FDI WORLD DENTAL CONGRESS 2024, İstanbul, Türkiye, 12 - 15 Eylül 2024, cilt.74, ss.223-224, (Özet Bildiri)
Özet
INTRODUCTION
Papillon-Lefèvre syndrome (PLS) is a rare syndrome caused by a mutation in the cathepsin C gene and is inherited as autosomal recessive. Studies have shown that the likelihood of developing the syndrome as a result of genetic mutations increases in children of parents who have had consanguineous marriages. It does not discriminate gender. Patients have hyperkeratotic areas on the hands and feet. It has been reported that teeth erupt normally but teeth are lost rapidly due to resorption in periodontal tissues. Prosthetic rehabilitation is very difficult due to aggressive resorption of the alveolar bone.
CASE DESCRIPTION
In 2021, a male and a female siblings visited the Gazi University Faculty of Dentistry, Department of Oral and Maxillofacial Surgery Clinic for dental treatment. After thorough examinations, the brother underwent total teeth extraction and subsequent prosthetic treatment, while the sister received a removable prosthesis after her teeth extraction. Two years later, it was determined that there was no issue with the brother's prosthesis, and he will continue to use it. As for the sister, a total prosthesis was planned after all her teeth were extracted. Patients who receive regular check-ups will be evaluated for dental implant applications once their growth and development are complete.
DISCUSSION
PLS is a very rare disorder. For this reason, literature information is also quite scarce. It is very important for dentists to be careful, to diagnose this syndrome early and to manage successful treatment.
CONCLUSION/CLINICAL SIGNIFICANCE
We aimed to report the effects of the rare PLS disease in the siblings