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Papillon-Lefévre syndrome.
Bagavad Gita Veerabahu1, Sc Chandrasekaran, Mohammed Nazish Alam
1Department of Periodontology, Sree Balaji Dental College and Hospital, Chennai, India.
Journal of Oral and Maxillofacial Pathology : JOMFP
|December 7, 2011
Summary
This case report details a 13-year-old male with Papillon-Lefévre syndrome, experiencing severe periodontitis and tooth mobility. Successful management required multidisciplinary care, including periodontal, prosthodontic, and dermatologic interventions.
Area of Science:
- Periodontology
- Genetics
- Dermatology
Background:
- Conventional periodontal therapy aims for tissue regeneration but faces challenges, particularly in syndromic cases.
- Papillon-Lefévre syndrome (PLS) is a rare genetic disorder characterized by periodontontoclasia and palmoplantar hyperkeratosis.
Observation:
- A 13-year-old male presented with mobile teeth, early primary dentition loss, and keratotic skin lesions.
- Radiographic findings revealed extensive bone loss and a "floating in air" appearance of teeth.
- Provisional diagnosis of Papillon-Lefévre syndrome was supported by clinical and radiographic evidence.
Findings:
- The patient was diagnosed with Papillon-Lefévre syndrome, a condition impacting periodontal health.
- Multidisciplinary management involving periodontal, prosthodontic, and dermatologic specialists was crucial for rehabilitation.
- A polymerase chain reaction assay was performed to assess virulence genes in aggressive periodontitis.
Implications:
- This case highlights the complexity of managing syndromic periodontitis.
- Integrated treatment approaches involving multiple specialties are essential for achieving functional and aesthetic rehabilitation in PLS patients.
- Long-term follow-up is necessary to maintain stable conditions after comprehensive treatment.
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