ReviewPostepy dermatologii i alergologii2020
Diagnosis of Papillon-Lefèvre syndrome: review of the literature and a case report.
Review in Postepy dermatologii i alergologii, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 26 citations in OpenAlex.
- Clinical characteristics of Papillon-Lefèvre syndrome: A KSA perspective.Journal of Taibah University Medical Sciences · 2025Review
- Triangular Lunulae in Papillon-Lefèvre Syndrome: A Case Report.Clinical case reports · 2025Article
- Oral Diseases as a Manifestation of Inborn Errors of Immunity.Journal of clinical medicine · 2024Review
- Papillon-Lefèvre syndrome in dental pediatric patient: A comprehensive review.The Saudi dental journal · 2024Review
- Dimethyl fumarate for treating Papillon-Lefèvre syndrome.JAAD case reports · 2023Article
- The first case report of Haim Munk disease with neurological manifestations and literature review.Clinical case reports · 2021Article
- Recurrent Aphthous Stomatitis: Treatment and Management.Dermatology practical & conceptual · 2021Review
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Papillon-Lefëvre syndrome (PLS), classified as ectodermal dysplasia, is an autosomal recessive condition related to the cathepsin C (CTSC) gene mutation. The first clinical symptoms, occurring most commonly between the ages of 1 and 4, are palmoplantar hyperkeratosis and also periodontitis resulting in the loss of most or all teeth in the same sequence in which they erupted. Most often the redness of palms and soles precede the occurrence of keratoderma. Moreover, excessive sweating, moderate mental retardation, the tendency to purulent skin and internal organs infection may occur. Lack of cathepsin seems to have a crucial role in the intensity of symptoms. In most of the patients, there can be observed impairment of phagocytosis and chemotaxis of neutrophils, granulocytes, leukocytes and cytotoxic lesion of fibroblasts and macrophages. Also, functional impairment of lymphocytes, neutrophils, and monocytes is observed. The study, using flow cytometry, showed a decreased percentage of T cells CD8+ and increased CD4:CD8 ratio.
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