ArticleJournal of Indian Society of Periodontology2013
Papillon-Lefevre syndrome: A case report of 2 affected siblings.
Article in Journal of Indian Society of Periodontology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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5 citing papers in PubMed, 13 citations in OpenAlex.
- Article
- The first case report of Haim Munk disease with neurological manifestations and literature review.Clinical case reports · 2021Article
- Papillon-Lefèvre syndrome: a series of five cases among siblings.Journal of medical case reports · 2016Article
- Papillon-Lefevre syndrome: Reporting consanguinity as a risk factor.The Saudi dental journal · 2014Article
- Papillon-Lefèvre syndrome.Anais brasileiros de dermatologiaArticle
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Authors and funding
3 authors at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
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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