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Keratosis palmoplantaris mutilans

Zuletzt geändert von Thomas Brinkmeier am 2025/03/16 15:37

Keratosis palmoplantaris mutilans

Syn: Pseudoainhum-artige Dermatose, Vohwinkel-Syndrom

Def: transgrediente Palmoplantarkeratose (PPK) mit Mutilationen und Innenohrschwerhörigkeit

Histr: Erstbeschreibung durch Vohwinkel im Jahre 1929

Man: in den ersten Lj.

KL: - diffuse Keratosen mit bienenwabenartiger Struktur

- warzenähnliche Keratosen an Hand- und Fußrücken, Ellbogen und Knien

- Uhrglasnägel

- Taubheit und sekundäre Sprachstörungen

- Hypogonadismus

Kopl: Ausbildung schmerzloser Schnürfurchen mit Spontanamputationen von Finger- oder Zehengliedern ("mutilans")

Lit:  

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Th: - Acitretin oral

CV: Osteogenese bei Kindern überwachen

- lokale Keratolytika

Stoff: Urea, Salizylsäure

Meth: Schmierseifenfußbäder, anschließend Hornhauthobel oder -raspel oder Bimsstein

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