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Papillon-Lefevre Syndrome In An Adolescent Female: A Case Study
M J Jijin1, H P Jaishankar2, Veena Sathya Narayaran3
1Post Graduate, Department of Oral Medicine and Radiology, Coorg Institute of Dental Sciences , Virajpet, Karnataka, India .
Journal of Clinical and Diagnostic Research : JCDR
|July 9, 2015
Summary
Papillon-Lefevre Syndrome (PLS) is a rare genetic disorder causing severe periodontitis and skin issues. Early dental and dermatological intervention is crucial for managing this condition.
Area of Science:
- Genetics
- Dermatology
- Dentistry
Background:
- Papillon-Lefevre Syndrome (PLS) is a rare autosomal-recessive disorder.
- Characterized by palmar-plantar hyperkeratosis, hyperhidrosis, and severe periodontitis.
- Key features include early-onset periodontitis, severe periodontal destruction, and Dural calcification.
Observation:
- A 14-year-old female presented with mobile teeth and dry, scaly skin on her feet, hands, and knees.
- The patient had a family history of consanguinity.
- Oral examination revealed generalized tooth mobility; physical examination noted dermatological lesions.
Findings:
- The patient was diagnosed with Papillon-Lefevre Syndrome (PLS).
- PLS manifests with a combination of distinct dermatological and dental abnormalities.
- Consanguinity was noted in one-third of PLS patients, as seen in this case.
Implications:
- Dentists play a critical role in the early diagnosis and management of PLS.
- Effective management requires a collaborative approach between dental and dermatological specialists.
- Early intervention and rehabilitation are essential for patients with PLS.
