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

Cases associated with this book:

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

Conference: DermatopathologyConsultations.com Teaching Collection