Work

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

Year: 2026

Type: article

Abstract: 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.

Source: BMC Oral Health

DOI: 10.1186/s12903-026-09034-7

Authors: Tingting Hu, Yuebo Wu, Xiaoyan Zou, Fang Ye

Institution: Nanchang University

Language: English

Topic: Oral microbiology and periodontitis research

Subfield: Periodontics

Field: Dentistry

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,190