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Nagelanomalien, angeborene

Zuletzt geändert von Thomas Brinkmeier am 2025/04/25 21:48

Nagelanomalien, angeborene

Etlg: - Trachyonychie

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Syn: Trachyonychia idiopathica, Zwanzig-Nägel-Dystrophie

Engl: twenty nail dystrophy, trachyonychia

Def: Sonderform der Onychodystrophie

Gen: autosomal-dominant

Vork: selten

Man: im frühen Kindesalter

Ass: Alopecia areata totalis

KL: dünne, brüchige Nagelplatte mit Längsstreifung und Glanzverlust

Prog: ggf. spontane Besserungstendenz im Alter

Th: - Calcipotriol-Betamethason-Salbe

Lit: Ann Dermatol. 2015 Aug;27(4):371-5 (Korea)

PT: CS (39 Pat.)

Appl: 1x/Tag (nicht okklusiv) für 6 Monate

- Acitretin

Lit: Indian J Dermatol Venereol Leprol. 2016 Nov-Dec;82(6):732-734

Co: Clobetasol topisch

Altn: Alitretinoin

Lit: J Eur Acad Dermatol Venereol. 2018 Apr 28. http://doi.org/10.1111/jdv.15024

PT: CS (21 Pat.)

- Triamcinolon-Matrixinjektionen

Lit: J Am Acad Dermatol. 2019 Oct 4. pii: S0190-9622(19)32809-9. http://doi.org/10.1016/j.jaad.2019.09.060

- Eigenplasma-Injektionen (PRP) in die Nagelmatrix

Lit: Dermatol Ther. 2019 Jan 18:e12831. http://doi.org/10.1111/dth.12831

- Cyclosporin A  

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- Nagel-Patella-Syndrom

Syn: Osteo-Arthro-Onycho-Dysplasie

Engl: nail-patella syndrome, hereditary osteo-onychodysplasia

Def: Sonderform der Onychodystrophie

Histr: Erstbeschreibung durch Chatelain im Jahre 1824

Gen: autosomal-dominant, Mutationen im LMX1B-Gen

Vork: selten

KL: - dystrophische Nägel

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Lok: Daumen und Zeigefinger sind Prädilektionsstellen

Note: Zehennägel sind i. d. R. nicht befallen

Bef: häufig Hyponychie, Onchychoschisis, dreieckige Lunula

Th: - Sililevo® (Fa. Taurus Pharma GmbH)

- Locycare® (Fa. Galderma Laboratorium GmbH)

- instabile Knie, da Kniescheibe hypoplastisch oder fehlend

Ass: - weitere orthopädische Anomalien

Bsp: Genu valgum, Pes equinovarus, exostotische Beckenhörner, hypoplastisches Radiusköpfchen, Hypoplasie der Skapula

- urogenitale Anomalien

Bef: Nierendysplasie, nephrotisches Syndrom, Goodpasture-Syndrom, chronische Pyelonephritis, Ureter duplex

- ophthalmologische Anomalien

Bef: Glaukom, Heterochromia iridis, Mikrokornea

Lit:  

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Th: orthopädische, ophthalmologische und nephrologische Abklärung

- Pachyonychia congenita

Def: Nagelverdickung im Rahmen des Jadassohn-Syndroms

- Leukonychia totalis

Bef: Weißfärbung aller Nagelplatten

DD: oberflächliche Dermatophytose der Nagelplatte

- kongenitale Großzehnageldystrophie

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Engl: congenital great toe nail dystrophy

- Genodermatosen mit Nagelanomalien

Bef: variabel

Bsp: Anonychie, Onychodystrophie

Lit: Our Dermatol Online. 2017;8(2):186-188.  

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KL: - Psoriasis vulgaris

- nichtbullöse kongenitale ichthyosiforme Erythrodermie

- Dyskeratosis follicularis (Darier, Morbus)

- Pemphigus familiaris benignus chronicus

- hidrotische ektodermale Dysplasie

- fokale dermale Hypoplasie

- Epidermolysis bullosa

- Dyskeratosis congenita

Note: Auch folgende Nagelanomalien können (ohne gleichzeitige Grunderkrankung) angeboren sein:

- Tüpfelnägel

- Quer- und Längsrillen

- Koilonychie 

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- Dystrophia mediana canaliformis

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- Uhrglasnägel

  
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