Synonym(s)
Dysplasia of nails with hypodontia; OMIM 189500; TNS; Tooth and nail syndrome
HistoryThis section has been translated automatically.
Witkop, 1975
DefinitionThis section has been translated automatically.
Rare autosomal dominant ectodermal dysplasia close to Clouston syndrome (Hidrotic ectodermal dysplasia) characterized by defects of the finger and toe nails(onychorrhexis, koilonychia) and hypodontia with normal hair structure and sweat gland function.
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EtiopathogenesisThis section has been translated automatically.
Heterozygous stop mutations in the home domain of the MSX1 gene are described.
TherapyThis section has been translated automatically.
Symptomatic.
LiteratureThis section has been translated automatically.
- Chitty LS et al (1996) Hidrotic ectodermal dysplasia of hair, teeth and nails: case reports and review. J Med Genet 33: 707-710
- Hudson CD, Witkop CJ Jr (1975) Autosomal dominant hypodontia with nail dysgenesis. Oral Surgery 39: 409-423
- Witkop CJ et al (1975) Hypoplastic enamel, onycholysis, and hypohidrosis inherited as an autosomal dominant trait: A review of ectodermal dysplasia syndromes. Oral Surgery 39: 409-421
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Outgoing links (4)
Ectodermal dysplasia ; Ectodermal dysplasia, hidrotic, autosomal dominant; Koilonychie; Onychorrhexis;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer