Ichthyosis


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ichthyosis

[‚ik·thē′ō·səs]
(medicine)
A congenital skin disease characterized by dryness and scales, especially on the extensor surfaces of the extremities.

Ichthyosis

 

fish skin disease, xeroderma, a skin disease characterized by sharply increased keratogenesis and retarded keratolysis.

Incidence of ichthyosis is a familial condition in half the cases and hereditary in 25 percent. It appears in early childhood (most often around age three), intensifies at puberty, and lasts throughout life. It is expressed in dryness of the skin and the accumulation of horny masses on the skin’s surface, in the form of scales or of massive horny plates that resemble fish scales. The excretion of oil and perspiration decreases sharply. The affection spreads over the entire skin surface, except at joint folds, axillae, and inguinal folds. On the face and scalp, ichthyosis appears in the form of furfuraceous desquamation. The condition of the skin improves in the summertime as a result of increased excretion of oil and sweat. Ichthyosis is treated with hot baths containing soda or table salt, followed by lubrication of the skin with emollient creams. Cod-liver oil and vitamins may be taken internally (in particular vitamin A). In the summer, prolonged sojourns in the south can be helpful (sea bathing).

I. N. VEDROVA

References in periodicals archive ?
Lamellar ichthyosis generally occurs at birth with a striking collodion-type membrane covering the body and underlying erythroderma, which then desquamates.
Mui Thomas is thought to be one of the oldest people known with Harlequin ichthyosis, as children with the condition often die, as they are vulnerable to infection as their skin cracks.
According to the First Skin Foundation, more than 16,000 babies are born with some form of ichthyosis annually.
Flake was forced to seek alternative childcare arrangements for her daughter, who wasn't on the trip, and to purchase lotion and additional clothing to conceal her Ichthyosis.
Clinically, the disease is presented with ichthyosis, hearing loss, hepatomegaly, splenomegaly, cirrhosis, cataract, myopathy, and mental retardation.
(5) Appearance of the skin of newborns with harlequin ichthyosis is characterized by erythematous diamond-shaped fissures and deep, thick, hyperkeratotic, brilliant-white plaques.
Keywords: Harlequin ichthyosis, autosomal dominant.
The dermatological examination was consistent with ichthyosis. Based on these clinical and laboratory findings, ARC syndrome was considered.
Every year, more than 16,000 babies are born with some form of ichthyosis. A recent study has determined that approximately 300 babies are born with a moderate to severe form of ichthyosis each year.
(NASDAQ: ALDX) has enrolled the first patient in a Phase 3 clinical trial of topical dermal reproxalap for the treatment of ichthyosis associated with Sjogren-Larsson Syndrome (SLS), a rare inborn error of metabolism caused by mutations in fatty acid aldehyde dehydrogenase, leading to ichthyosis, neurological disorders, and retinal disease, the company said.
The mum-of-three, of Chattaroy, Washington, USA, said: "I had never heard of Harlequin ichthyosis before the doctor took me aside and showed me a text book with pictures of babies with this condition.