- 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
- 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