Generalisierte erythrosquamöse Dermatose

A 21-year-old man presented with generalized erythema, erosions and hyperkeratoses since birth. Histology revealed epidermolytic hyperkeratosis with degeneration of the upper epidermis and perinuclear deposits of abnormal keratin aggregations. Epidermolytic ichthyosis was diagnosed. This congenital...

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Hauptverfasser: Weberschock, Tanja (VerfasserIn) , Haußer-Siller, Ingrid (VerfasserIn) , Gholam, Patrick (VerfasserIn) , Hartmann, Martin (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Deutsch
Veröffentlicht: 07 Janauary 2012
In: Der Hautarzt
Year: 2012, Jahrgang: 63, Heft: 1, Pages: 47-51
ISSN:1432-1173
DOI:10.1007/s00105-011-2277-8
Online-Zugang:Verlag, Volltext: https://doi.org/10.1007/s00105-011-2277-8
Volltext
Verfasserangaben:T. Weberschock, I. Hausser, P. Gholam, M. Hartmann
Beschreibung
Zusammenfassung:A 21-year-old man presented with generalized erythema, erosions and hyperkeratoses since birth. Histology revealed epidermolytic hyperkeratosis with degeneration of the upper epidermis and perinuclear deposits of abnormal keratin aggregations. Epidermolytic ichthyosis was diagnosed. This congenital Ichthyosis occurs due to mutations of keratin 1 or 10 genes that leads to defects of intra- and intercellular structural integrity in the spinous and granular layers with compensatory hyperkeratosis. After childhood, life expectancy is normal but lifelong therapeutic and skin care measures are required.
Beschreibung:Gesehen am 04.04.2019
Beschreibung:Online Resource
ISSN:1432-1173
DOI:10.1007/s00105-011-2277-8