Journal of Indian Society of Periodontology
Journal of Indian Society of Periodontology
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CASE REPORT
Year : 2013  |  Volume : 17  |  Issue : 3  |  Page : 373-377

Papillon-Lefevre syndrome: A case report of 2 affected siblings


1 Department of Dentistry, Dr. R. P. Government Medical College, Kangra at Tanda, Himachal Pradesh, India
2 Department of Periodontics, National Dental College, Derabassi, Punjab, India
3 Department of Medicine, Dr. R. P. Government Medical College, Kangra at Tanda, Himachal Pradesh, India

Correspondence Address:
Anupriya Sharma
Department of Dentistry, Dr. R. P. Government Medical College, Kangra at Tanda - 176 001 Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-124X.115643

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Papillon-Lefèvre syndrome (PLS) is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome, but a recent report has suggested that the condition is linked to x mutations of the cathepsin C gene. The purpose of this report is to describe two cases of PLS in the same family who presented to the Department of Dentistry of Dr. R. P. Government Medical College at Tanda, Kangra (Himachal Pradesh) with a chief complaint of mobility and rapid loss of teeth. Hyperkeratosis of palms and soles was present. On intraoral examination, there was severe gingival inflammation, abscess formation, and deep periodontal pockets with mobility of teeth. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS. The dental treatment comprised oral prophylaxis, scaling and root planning, antibiotic therapy, instructions on oral hygiene, restorations, extraction of hopelessly affected teeth, and prosthetic rehabilitation.


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