Papillon-Lefevre Syndrome In An Adolescent Female: A Case Study
Papillon-Lefevre Syndrome (PLS) is a rare inherited autosomal-recessive condition with one-third of the patients’ showing consanguinity of the parents. Lesions are characterised by palmar-plantar hyperkeratosis and hyperhidrosis. Early onset of periodonditis, severe periodontal destruction in both...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Private Limited
2015-05-01
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| Series: | Journal of Clinical and Diagnostic Research |
| Subjects: | |
| Online Access: | https://jcdr.net/articles/PDF/5921/12780_CE[Ra1]_F(AK)_PF1(PAG)_PFA(P)_PF2(PAG).pdf |
| Summary: | Papillon-Lefevre Syndrome (PLS) is a rare inherited autosomal-recessive condition with one-third of the patients’ showing consanguinity
of the parents. Lesions are characterised by palmar-plantar hyperkeratosis and hyperhidrosis. Early onset of periodonditis, severe
periodontal destruction in both primary and permanent dentitions, and calcification of the duramater form the three important features
of this disease. Here, we present a case of a 14-year-old female who presented to the Department of Oral Medicine and Radiology
with a complaint of mobility of the teeth since four months. Oral examination of the patient showed generalised mobility of the teeth.
General physical examination of the patient showed dry scaly skin on dorsum of bilateral feet, hands, and knee. The patient had familial
history positive for consanguinity. The patient was medically diagnosed as positive for PLS. The patients with PLS show combination of
dermatological and dental lesions and it requires the dentist to assume a more prominent role in early treatment and rehabilitation. There
is a need for symbiotic and synergetic approach between the two specialties for effective management of this rare disease. |
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| ISSN: | 2249-782X 0973-709X |