ArticleCase reports in dentistry2019
Papillon-Lefèvre Syndrome: Diagnosis, Dental Management, and a Case Report.
Article in Case reports in dentistry, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 22 citations in OpenAlex.
- Papillon-Lefèvre Syndrome: Case Report of Two Sisters.International journal of clinical pediatric dentistry · 2025Article
- Papillon-Lefèvre syndrome in dental pediatric patient: A comprehensive review.The Saudi dental journal · 2024Review
- Recent developments in the diagnosis, treatment, and management of Papillon-Lefèvre Syndrome.Evidence-based dentistry · 2024Article
- Diagnosis and Management of Papillon-Lefevre Syndrome: A Rare Case Report and a Brief Review of Literature.Cureus · 2023Article
- Multidisciplinary management of Papillon-Lefevre syndrome as a result of consanguineous marriage.BMJ case reports · 2022Article
- Oral Mucosa and Nails in Genodermatoses: A Diagnostic Challenge.Journal of clinical medicine · 2021Review
- The first case report of Haim Munk disease with neurological manifestations and literature review.Clinical case reports · 2021Article
- Papillon-Lefèvre syndrome (PLS) with novel compound heterozygous mutation in the exclusion and Peptidase C1A domains of Cathepsin C gene.Molecular biology reports · 2020Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
aimThis paper revisits Papillon-Lefèvre syndrome (PLS), addresses its diagnostic update and dental management, and reports a case of a 5-year-old Lebanese patient with consanguineously married parents.
backgroundPLS, also known as "keratoris palmoplantaris with periodontopathia" and "hyperkeratosis palmoplantaris with periodontosis," is an extremely rare autosomal-recessive trait that combines a diffuse palmoplantar hyperkeratosis and a severe generalized, progressive prepubertal form of a precocious form of juvenile, aggressive periodontitis. CASE DESCRIPTION: We are reporting a 5-year-old boy that sustained a spontaneous loss of all his primary teeth. At consultation, he was under treatment for hyperkeratosis of his palms and soles. Detailed family history of the child revealed that the patient's parents, grandparents, and relatives were consanguineously married and two of his cousins displayed similar clinical signs (palmoplantar hyperkeratosis and premature loss of deciduous and most of the permanent teeth).
conclusionPLS is an extremely rare disorder that usually becomes apparent from approximately 1-5 years of age. Genetic counseling should always be suggested to parents of affected children, informing them of chances of their offspring having the inherited disease.
Identifiers
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.