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Papillon-Lefèvre syndrome: a case report
1Department of Periodontics, Government Dental College and Hospital, Fort, Bangalore, India. drarpradeep@hotmail.com
The New York State Dental Journal
|February 7, 2009
Summary
Papillon-Lefèvre syndrome, a rare genetic disorder, causes severe periodontal disease and skin issues. Early, intensive treatment combining mechanical and antimicrobial approaches may help manage its progression.
Area of Science:
- Genetics and rare diseases
- Dermatology
- Periodontology
Background:
- Papillon-Lefèvre syndrome is a rare autosomal recessive disorder.
- Characterized by severe early-onset periodontitis and palmoplantar hyperkeratosis.
- Increased susceptibility to infections is also a common feature.
Observation:
- An 8-year-old child presented with classic symptoms of Papillon-Lefèvre syndrome.
- The patient exhibited periodontal disease in both primary and permanent teeth, alongside palmar and plantar keratosis.
- Family history indicated consanguinity and affected siblings, supporting a genetic etiology.
Findings:
- The case highlights the typical clinical presentation of Papillon-Lefèvre syndrome.
- Review of literature suggests potential for disease modification.
- Successful outcomes reported in some patients treated with combined therapies.
Implications:
- Early diagnosis and intervention are crucial for managing Papillon-Lefèvre syndrome.
- Intensive mechanical and antimicrobial periodontal therapy may halt disease progression.
- Further research into optimal treatment strategies for this rare syndrome is warranted.
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