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Periodontitis associated with Papillon-Lefèvre syndrome
E Bimstein1, J Lustmann, M N Sela
1Department of Pediatric Dentistry, Hadassah Faculty of Dental Medicine, Hebrew University, Jerusalem, Israel.
Journal of Periodontology
|June 1, 1990
Summary
Papillon-Lefèvre Syndrome (PLS) involves specific bacteria like Actinobacillus actinomycetemcomitans and immune dysfunction. This study highlights their role in the disease progression of a young patient.
Area of Science:
- Periodontology
- Immunology
- Genetics
Background:
- Papillon-Lefèvre Syndrome (PLS) is a rare genetic disorder characterized by palmoplantar hyperkeratosis and severe periodontitis.
- Understanding the microbial and host factors contributing to PLS pathogenesis is crucial for effective management.
Observation:
- A 14-year-old female patient with PLS presented with distinct subgingival microflora.
- Elevated levels of Actinobacillus actinomycetemcomitans and surface translocating bacteria were identified.
- Significantly increased antibody levels against five bacterial species, including persistent high levels against A. actinomycetemcomitans post-antibiotics, were observed.
Findings:
- The patient exhibited polymorphonuclear leukocyte (PMN) dysfunction, with significantly increased O2 release compared to controls.
- A strong association between A. actinomycetemcomitans colonization and PMN dysfunction was noted.
- Immune response patterns suggest a specific susceptibility in PLS patients.
Implications:
- The findings support a significant role for A. actinomycetemcomitans and PMN dysfunction in the development of PLS.
- This research may inform targeted therapeutic strategies focusing on microbial control and immune modulation in PLS patients.
- Further investigation into the interplay between genetics, microbial agents, and immune responses in PLS is warranted.