Autosomal dominant ichthyosis exfoliativa.
An unusual ichthyosiform dermatosis with circumscribed areas of blistering hyperkeratoses as well as scaly areas was observed in five consecutive generations of a German family. Light and electron microscopical studies revealed oedema of the keratinocytes in the upper epidermis but no defects of tonofilaments and keratohyalin granules. We suggest that this type of ichthyosis exfoliativa inherited as an autosomal dominant trait represents a new and distinct entity.
Vakilzadeh
F
F
Department of Dermatology, General Hospital of Hildesheim, Germany.
Kolde
G
G
D000328:Adult; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D007057:Ichthyosis / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D012867:Skin / Q000473:pathology