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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.

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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.

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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.