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epidermolytic hyperkeratosis
(redirected from Congenital bullous ichthyosiform erythroderma)

   Also found in: Wikipedia 0.02 sec.
hyperkeratosis /hy·per·ker·a·to·sis/ (-ker″ah-to´sis)
1. hypertrophy of the stratum corneum of the skin, or any disease so characterized.
2. hypertrophy of the cornea.

epidermolytic hyperkeratosis  a hereditary disease, with hyperkeratosis, blisters, and erythema; at birth the skin is entirely covered with thick, horny, armorlike plates that are soon shed, leaving a raw surface on which scales then reform.
hyperkeratosis follicula´ris in cu´tem pe´netrans  Kyrle's disease.

epidermolytic hyperkeratosis
Etymology: Gk, epi + derma, skin + lysis, loosening; Gk, hyper, excess + keras, horn + osis, condition
a rare autosomal-dominant form of ichthyosis with a high frequency of spontaneous mutations. Present at birth, it is characterized by generalized erythroderma and severe hyperkeratosis with small wartlike scales over the entire body, especially in body folds, and sometimes on the palms and soles. There are also recurrent bullae on the lower limbs. If sepsis and electrolyte imbalances are not treated correctly in neonates, morbidity can occur. Also called bullous congenital ichthyosiform erythroderma. See also ichthyosis.

hyperkeratosis
1. hypertrophy of the horny layer (stratum corneum) of the skin, or any disease characterized by it; the hyperkeratoses may have distinctive formats, e.g. annular (ring formations), basket-weave, compact, laminated.
2. hypertrophy of the cornea.

bovine hyperkeratosis
chlorinated naphthalene poisoning.
digital hyperkeratosis
increased thickness of the keratinized epidermis of footpads in dogs and rarely cats. May be in response to trauma or associated with distemper (hardpad disease), or pemphigus foliaceus.
epidermolytic hyperkeratosis
a form of ichthyosis in humans which is inherited as an autosomal dominant trait; there is severe degeneration of the granular layer of the epidermis.
juvenile hyperkeratosis
a crusting dermatosis over bony prominences, face and chin of young dogs. See zinc-responsive dermatosis.
nasal hyperkeratosis
an abnormal thickening, sometimes with fissures, of the planum nasale of dogs. May occur in association with digital hyperkeratosis (see above) as a feature of distemper (hardpad disease). Also seen in pemphigus foliaceus and discoid lupus erythematosus.
Enlarge picture
Nasal hyperkeratosis. By permission from Kummel BA, Color Atlas of Small Animal Dermatology, Mosby, 1989
nasodigital hyperkeratosis
see nasal hyperkeratosis, digital hyperkeratosis (above).
orthokeratotic hyperkeratosis
hyperkeratosis with non-nucleated cells present.
parakeratotic hyperkeratosis
hyperkeratosis with nucleated cells present; called also parakeratosis.


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