Long-term periodontal maintenance in a patient with Papillon-Lefèvre syndrome: an 8-year case report.

BACKGROUND

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder with a prevalence of approximately 1-4 cases per one million individuals. This condition frequently leads to premature loss of both the primary and the permanent teeth. PLS is primarily caused by mutations in the cathepsin C (CTSC) gene, resulting in the loss of CTSC function, which disrupts immune regulation and triggers severe inflammatory responses. Maintaining and preserving teeth in these patients therefore presents a significant challenge for periodontists.

CASE PRESENTATION: The patient received multidisciplinary treatment involving periodontics, oral and maxillofacial surgery, and prosthodontics. Hopeless teeth were extracted, removable dentures were provided, and supportive periodontal maintenance was performed over an 8-year period. Adjunctive systemic antibiotics were used during active treatment and selected maintenance visits because of severe inflammation and multiple sites with suppuration, but this approach was not designed as a standardized long-term antibiotic protocol.

RESULTS

At the latest follow-up, 22 natural teeth were retained. The bleeding on probing rate decreased from 71% at baseline to 18% in March 2025, the proportion of sites with probing depth ≥ 4 mm decreased from 54 to 27%, and the Plaque Index improved from 2-3 to 1-2. Clinical attachment level was not consistently recorded and therefore could not be analyzed longitudinally.

CONCLUSION

This single case suggests that early diagnosis, extraction of hopeless teeth, individualized multidisciplinary periodontal-prosthodontic management, regular supportive periodontal care, sustained plaque control, and strong family cooperation may help prolong the retention of functional natural teeth in selected patients with Papillon-Lefèvre syndrome. Further longitudinal studies with standardized periodontal, microbiological, and antimicrobial-resistance monitoring are needed.