Ichthyosis
| Definition: |
Any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. Most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome. |
| Notes: |
GEN or unspecified; prefer specifics; unspecified dryness of skin is indexed under SKIN DISEASES, not here |
| Also Called: |
Xeroderma |
Ichthyosis Categories.
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Ichthyosiform Erythroderma, Congenital - Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type. |
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Ichthyosis Vulgaris - Most common form of ICHTHYOSIS characterized by prominent scaling especially on the exterior surfaces of the extremities. It is inherited as an autosomal dominant trait. |
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Ichthyosis, X-Linked - Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency. |
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Sjogren-Larsson Syndrome - Rare syndrome of autosomal recessive inheritance characterized by ichthyosis, mental retardation, and spastic pyramidal symptoms. It is associated with a defect in fatty alcohol metabolism. |
Ichthyosis Definitions and Terms
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