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Olmsted-Syndrom

Zuletzt geändert von Thomas Brinkmeier am 2023/08/04 01:08

Olmsted-Syndrom

Engl: Olmsted syndrome

Def: hereditärer Defekt der Keratinisierung

Histr: Erstbeschreibung durch Olmsted im Jahre 1927

KL: - mutilierende Palmoplantarkeratose (PPK)

Bef: - scharf begrenzte hyperkeratotische Plaques

- Konstriktion der Finger und Zehen

Kopl: Spontanamputationen der distalen Phalangen

- periorale Hyperkeratose/hyperkeratotische Plaques in Umgebung der Körperöffnungen

- Leukokeratose

- Onychodystrophie

- Alopezie

Ass: - palmoplantare Hyperhidrosis

- kongenitale Taubstummheit

Hi: - Orthohyperkeratose

- Papillomatose

- Akanthose

IHC: - abnormale Expression von Keratin 5 + 14 ähnlich wie bei anderen hyperproliferativen Erkrankungen

- erhöhte Expression des Proliferationsmarkers Ki-67 in den basalen und suprabasalen Keratinozyten

Lit: - Case Rep Dermatol Med. 2012;2012:927305.  

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- JAAD Case Rep. 2022 Jun 7;25:83-85. http://doi.org/10.1016/j.jdcr.2022.05.029

- JAAD Case Rep. 2022 Jun 7;25:83-85. http://doi.org/10.1016/j.jdcr.2022.05.029

Th: - Versuch mit Acitretin in niedriger Dosis

- Glukokortikoide

- Keratolytika

- Erlotinib

Lit: JAMA Dermatol. 2020 Jan 2. http://doi.org/10.1001/jamadermatol.2019.4126

- operative Maßnahmen im Bereich der Akren

  
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