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What is Ichthyosis?

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What is Ichthyosis?

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Definition Derived from two Greek words meaning “fish” and “disease,” ichthyosis is a congenital (meaning present at birth) dermatological (skin) disease that is represented by thick, scaly skin. Description The ichthyoses are a group of skin diseases caused by an abnormality in skin growth that results in drying and scaling. There are at least 20 types of ichthyosis. Ichthyosis can be more or less severe, sometimes accumulating thick scales and cracks that are painful and bleed. Ichthyosis is not contagious. Some forms of ichthyosis are inherited while others are acquired in later life as a symptom of systemic disorders. The most common form of ichthyosis, accounting for 95% of all cases of ichthyosis, is called ichthyosis vulgaris (vulgaris is the Latin word for “common”), and occurs in approximately one person in every 250. It is inherited in an autosomal dominant manner. The most rare types of ichthyosis occur in fewer than one person in one million and are inherited in an autosoma

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• Ichthyosis: Fish-like scaly dry skin areas. • Ichthyosis: 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. Source – Diseases Database • Ichthyosis: any of several congenital diseases in which the skin is fishlike (dry and scaly). Source – WordNet 2.1 Ichthyosis is listed as a “rare disease” by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Ichthyosis, or a subtype of Ichthyosis, affects less than 200,000 people in the US population.

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Ichthyosis is a genetic skin disorder. It is characterized by dry skin that builds up and flakes off. The skin can sometimes appear to be scales, which is where the name originates; ichthys means “fish” in ancient Greek. There are several types of genetic ichthyosis and each presents in a slightly different fashion. These types are ichthyosis vulgaris, lamellar ichthyosis, epidermolytic hyperkeratosis, X-linked ichthyosis, and congenital ichthyosiform erythroderma. Ichthyosis vulgaris accounts for 95% of cases, making it the most common form. It is usually found on the trunk and legs and makes its appearance at puberty. Epidermolytic hyperkeratosisis is seen as red, moist skin at birth. Lemellar Ichthyosis is an extremely rare version where babies are born with a mucus membrane over their skin and the entire body sheds. X-linked ichthyosis is found on the trunk and extremities and is present at birth. Congenital ichthyosiform erythroderma is the mildest of the forms of the disease and

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Ichthyosis is characterised by persistently dry, thickened, rough, fish scale skin. There are at least 20 varieties of ichthyosis, including inherited and acquired forms. Ichthyosis vulgaris, accounts for 95% of all ichthyosis cases. Inheritance is autosomal dominant i.e. it is passed from a parent to about half their children. Signs and symptoms of ichthyosis vulgaris usually become apparent within the first year of life. The other main forms of inherited ichthyosis include lamellar ichthyosis, epidermolytic hyperkeratosis, and X-linked ichthyosis. Acquired ichthyosis is not inherited and occurs for the first time in adulthood. It is usually associated with some general systemic disease, such as underactive thyroid states, sarcoidosis, lymphoma, generalised cancer or HIV infection. It may be provoked by certain medications (such as kava, nicotinic acid and hydroxyurea).

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Posted by alexg4 on Thursday November 6, 2008 at 7:54 AM and tagged with biology, definition, genetics, ichthyosis, science.

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