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Papillon-Lefevre syndrome.

V P Singh¹, A Sharma, S Sharma

¹Department of Orthodontics, College of Dental Sciences, BPKIHS, Dharan, Nepal. varundc@gmail.com

Mymensingh Medical Journal : MMJ

|November 15, 2011

Summary

This summary is machine-generated.

Papillon-Lefevre syndrome, a rare genetic disorder, causes severe palmoplantar hyperkeratosis and precocious tooth loss due to periodontal destruction. This case highlights typical symptoms and immunologic findings in a patient with this syndrome.

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Area of Science:

Genetics
Immunology
Dermatology

Background:

Papillon-Lefevre syndrome is an autosomal recessive disorder.
It is characterized by palmoplantar hyperkeratosis and severe, aggressive periodontitis.
The exact pathogenesis remains unclear, with proposed immunologic, microbiologic, and genetic factors.

Observation:

This report details a case of Papillon-Lefevre syndrome.
The patient presented with generalized plaque, halitosis, mobile teeth, and periodontal pockets with pus.
Clinical presentation included significant destruction of alveolar bone.

Findings:

Blood and biochemical reports were within normal limits.
Immunological assessment revealed low CD3+ and CD4+ T-cell counts.
These findings suggest a potential immunodeficiency component in this patient.

Implications:

Early diagnosis and management are crucial for preserving dentition.
Understanding the immunologic basis may lead to targeted therapies.
This case contributes to the literature on Papillon-Lefevre syndrome pathogenesis.