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Papillon–Lefèvre Syndrome in the Permanent Dentition: A Rare Case Report
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Hakimeh Ahadian1    , Arezoo Heidary *2 |
1- Shahid Sadoughi University of Medical Sciences
2- Shahid Sadoughi University of Medical Sciences , arezooheidary84@yahoo.com |
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Abstract: (4 Views) |
Background and Aim: Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disease associated with hyperkeratosis of the palms and soles and premature destruction of the periodontium of primary and permanent teeth. The disease is caused by mutations in the gene encoding cathepsin C. Retinoids are used to treat skin lesions. In cases of intraoral involvement, despite extensive periodontal involvement, antibiotic treatment may sometimes be somewhat effective..
Case report: A 17-year-old female patient presented with complaints of loose permanent teeth. According to the history obtained from the patient's mother, the primary teeth exfoliated at the appropriate time. On clinical examination, moderate to severe generalized tooth mobility was observed in all teeth. The patient's palms and soles also showed moderate hyperkeratosis. The patient was prescribed oral antibiotics, including amoxicillin and metronidazole, along with chlorhexidine 0.12% mouthwash for two weeks.
Conclusion: Given the progressive course and poor prognosis of the disease, and since dentists are among the first clinicians who may diagnose this syndrome, obtaining a detailed history and performing a complete examination in children with premature tooth loosening are important steps toward timely diagnosis and slowing disease progression through appropriate clinical measures. |
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| Keywords: Papillon-Lefevre syndrome, tooth loss, hyperkeratosis, palms and soles |
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Type of Study: case report |
Subject:
Dentistry
Received: 2025/05/24 | Accepted: 2026/11/8
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