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Papillon Lefevre syndrome.
Shibu Thomas1, Sarat Gummadapu, Auswaf Ahsan
1Department of Oral Medicine and Radiology, College of Dental Surgery, Manipal-576119, Karnataka, India. drshibuthomas@rediffmail.com.
Saudi Medical Journal
|July 6, 2004
Summary
Papillon-Lefevre syndrome, a rare genetic disorder, causes severe palmoplantar hyperkeratosis and early-onset periodontitis, resulting in premature tooth loss. This report details a case and discusses potential treatment strategies for this condition.
Area of Science:
- Genetics
- Dermatology
- Dentistry
Background:
- Papillon-Lefevre syndrome is an extremely rare autosomal recessive disorder.
- It is characterized by distinct clinical manifestations affecting the skin and oral cavity.
Observation:
- The syndrome presents with pronounced palmoplantar hyperkeratosis, a thickening of the skin on the palms and soles.
- Severe, early-onset periodontitis is a hallmark, leading to rapid destruction of the periodontium.
Findings:
- The case report focuses on a patient diagnosed with Papillon-Lefevre syndrome.
- The report includes observations on the progression of the condition and its impact on dentition.
Implications:
- Understanding Papillon-Lefevre syndrome is crucial for early diagnosis and management.
- This case highlights the importance of a multidisciplinary approach to treatment, involving dermatological and dental interventions.
- Further research into effective treatment strategies for Papillon-Lefevre syndrome is warranted to improve patient outcomes and prevent tooth loss.