Combined therapy in a patient with Papillon-Lefèvre syndrome: a 13-year follow-up.

BACKGROUND

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disease characterized by hyperkeratosis of the palms and soles combined with premature loss of the primary and permanent dentition. Several treatment regimens have been recommended in the literature; however, a definitive treatment protocol has not been established. This case report evaluates the success of combined therapy in managing a patient with PLS.

METHODS

A 6-year-old girl diagnosed with PLS presented with aggressive periodontal destruction of her primary and permanent dentitions. After extraction of periodontally affected teeth, the edentate region was rehabilitated with different temporary dentures until her skeletal growth was complete. At the same time, her orthodontic treatment was performed. The early loss of her incisors resulted in inadequate alveolar bone height and width for esthetic-advanced prosthetic rehabilitation. Alveolar bone augmentation was performed, and 6 months later, two intraosseous dental implants were placed into the central incisor zone.

RESULTS

After 13 years of treatment and follow-up, the patient had periodontally healthy permanent dentition. She had practiced meticulous oral hygiene, and the orthodontic treatment was successful and without incident. Alveolar ridge augmentation and placement of an intraosseous implant with guided bone regeneration were performed successfully.

CONCLUSIONS

This case report demonstrates that individually developed treatment protocols can provide long-term dental/periodontal success in patients with PLS. A multidisciplinary approach with advanced periodontal surgery, orthodontic and prosthetic treatment, and implant therapy may be an appropriate treatment modality for dental rehabilitation in patients with PLS.