Structured Summary
Abstract
Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease.
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Synonyms
9 entry terms
- Keratosis Palmoplantar Periodontopathy
- Keratosis Palmoplantaris with Periodontopathia
- Papillon Lefevre Disease
- Papillon-Lefevre Syndrome
- Keratosis Palmoplantar Periodontopathies
- Palmoplantar Periodontopathies, Keratosis
- Papillon Lefevre Syndrome
- Haim-Monk Syndrome
- Haim Monk Syndrome
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Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
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History Note
91(75); was see under KERATOSIS PALMARIS ET PLANTARIS 1975-90 (which became KERATODERMA, PALMOPLANTAR 1993)
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References
- National Library of Medicine. Papillon-Lefevre Disease. Medical Subject Headings (MeSH). 2026. Unique ID D010214. http://id.nlm.nih.gov/mesh/2026/D010214
- Papillon-Lefevre Disease. In: Wikipedia. https://en.wikipedia.org/wiki/Papillon%E2%80%93Lef%C3%A8vre_syndrome
- Papillon-Lefevre Disease. In: Wikidata. https://www.wikidata.org/wiki/Q2050791