Epidermolytic hyperkeratosis

    Clinical Features:

    • Autosomal dominant disorder presenting at birth with widespread blistering followed by generalized ichthyosis characterized by brown verrucous scales
    • Caused by a defect in either the keratin 1 or keratin 10 genes

    Histologic Features:

    • Compact hyperorthokeratosis
    • Vacuolization of the superficial keratinocytes with large keratohyalin granules and trichohyalin granules
    • Focal blister formation may be present where the vacuolated keratinocytes have coalesced.
    • Psoriasiform hyperplasia in later lesions
    External Links:


    Cases associated with this book:

  • Epidermolytic hyperkeratosis
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: DermatopathologyConsultations.com Teaching Collection