Journal
HAUTARZT
Volume 63, Issue 1, Pages 47-51Publisher
SPRINGER
DOI: 10.1007/s00105-011-2277-8
Keywords
Ichthyosis; Epidermolytic hyperkeratosis; Acanthokeratolysis; Keratin; Genetic skin disease
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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.
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