Multidisciplinary rehabilitation of Papillon-Lefèvre syndrome using zygomatic implants: A 3-year case report.

Abstract

Background: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar keratoderma and rapidly progressive periodontitis, resulting in early tooth loss and severe alveolar bone resorption. Prosthetic rehabilitation in such patients remains a significant clinical challenge due to compromised bone support and functional limitations. This report aims to evaluate the effectiveness of zygomatic implants as part of a multidisciplinary approach for oral rehabilitation in a patient with PLS.

Methods: A 17-year-old female with a confirmed diagnosis of PLS presented with partial edentulism and notable psychosocial impairment. Comprehensive clinical and radiographic evaluation informed a staged, multidisciplinary treatment approach. Phase I therapy included thorough periodontal treatment followed by extraction of hopeless teeth was performed. Rehabilitation of the severely atrophic maxilla was achieved using zygomatic implants, while conventional endosseous implants were placed in the mandible. A full-arch implant-supported fixed hybrid prosthesis was subsequently fabricated and delivered. The patient was maintained on a structured follow-up protocol and evaluated over a 3-year period, demonstrating favorable clinical outcomes.

Results: At the 3-year follow-up, the implants demonstrated excellent stability with no signs of peri-implant pathology. The prosthesis provided satisfactory esthetic and functional outcomes, significantly improving masticatory efficiency and speech. The patient also exhibited marked improvement in psychological well-being and quality of life.

Conclusions: Zygomatic implants represent a reliable and effective treatment modality for rehabilitating patients with severe maxillary atrophy associated with PLS. A multidisciplinary, patient-centered approach is critical for achieving predictable long-term functional, esthetic, and psychosocial outcomes in complex syndromic cases.

Key points: Zygomatic implants can be a reliable option to restore function and esthetics when the upper jaw is severely resorbed in Papillon-Lefèvre syndrome. Before considering implants in syndromic periodontitis, it is crucial to first control the periodontal condition and stick to a strict, long-term maintenance plan. In young patients with complex periodontal issues, a coordinated team approach can meaningfully improve chewing, appearance, and overall confidence and quality of life.

Plain language summary: Papillon-Lefèvre syndrome is a rare inherited condition that causes severe gum disease and thickening of the skin on the hands and feet. Many patients lose their teeth at a very young age because of rapid destruction of the supporting bone around the teeth. Restoring oral function in these patients is difficult because there is often very little bone remaining in the jaws for conventional dental implants. This report describes the treatment of a 17-year-old female with Papillon-Lefèvre syndrome using a multidisciplinary approach involving periodontal therapy, tooth extraction, zygomatic implants, conventional dental implants, and full-mouth prosthetic rehabilitation. Initial treatment focused on controlling infection and stabilizing the gums before definitive rehabilitation was performed. Zygomatic implants were used in the upper jaw because of severe bone loss. The patient was followed for 3 years after treatment. The implants remained stable, and the final prosthesis improved chewing ability, speech, appearance, and psychological confidence. This case demonstrates that careful planning, strict maintenance, and a multidisciplinary approach can achieve successful long-term rehabilitation in patients with severe syndromic periodontal destruction.