Xeroderma Pigmentosum


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xeroderma pigmentosum

[‚zir·ə′dər·mə ‚pig·mən′tō·səm]
(medicine)
A genodermatosis characterized by premature degenerative changes in the form of keratoses, malignant epitheliomatosis, and hyper- and hypopigmentation.

Xeroderma Pigmentosum

 

a chronic congenital disease of the skin, first described by the Austrian physician M. Kaposi in 1870.

Xeroderma pigmentosum is a hereditary disease (a recessive genodermatosis) often found in children whose parents are blood relatives. The disease begins in early childhood (at age two or three) with an increased sensitivity of the skin to ultraviolet rays. In the spring, reddish spots (to the size of a bean) appear on the exposed areas of the body (face, neck, hands, forearms, legs, and feet). These later acquire a yellowish brown tint, resembling freckles. The affected skin gradually becomes dry. Small scales form on its surface, sometimes accompanied by cracks and a purulent crust (a result of associated secondary infection). The skin in the affected areas subsequently becomes thin and begins to wrinkle. Distended blood vessels appear on its surface (telangiectases). Depigmented areas appear along with the pigmented spots. Often, warty growths appear around the nidi; these may degenerate into basal-cellular or spino-cellular epitheliomas. For this reason, xeroderma pigmentosum is regarded as a precancerous disease.

The treatment of xeroderma pigmentosum is directed mainly against the tumorous and ulcerous formations, using corticosteroids, short-focus X-ray therapy, electric coagulation, and surgical intervention.

Photodesensitizing preparations, photo-protective creams (containing 10 percent phenyl salicylate and 5 percent quinine), and the salve Fotonem are helpful in preventing appearances of the condition. Exposure to the sun should be avoided (especially during the spring and summer). Regular checkups are recommended.

I. IA. SHAKHMEISTER

References in periodicals archive ?
Xeroderma pigmentosum is characterized by early onset photosensitivity, photophobia, and multiple freckles.
Low synthesis of retinoic acid due to impaired cytochrome P450 1a1 expression in mouse xeroderma pigmentosum fibroblasts.
These patients did not have a diagnosis of Xeroderma pigmentosum (XP) or any other genetic defects associated with an impairment of DNA repair capacity.
Dale's loss of hearing is just one of the problems related to Xeroderma Pigmentosum (XP), the condition from which both he and his older sister, Saicha, 14, suffer.
The film's plot centers on a young mother, Grace, and her two children, Anne and Nicholas, whose isolation in their cavernous mansion is overdetermined by several factors: its location on the English island of Jersey in 1945, the fog that perpetually shrouds it, and the children's disease, Xeroderma Pigmentosum, which necessitates keeping them out of the light.
Other risk factors associated with melanoma include family or personal history of melanoma, multiple melanocytic nevi (atypical or dysplastic moles), advancing age, giant congenital melanocytic nevi, xeroderma pigmentosum, and chronic immunosupresssion (Volker, 2004).
They help prevent not only sunburn, but also brief daily UV exposures that cumulatively accelerate skin aging and increase skin cancer risks, ha addition, the films can be lifesavers for people with dangerous photosensitive conditions such as xeroderma pigmentosum.
It also teaches the reader about medicinal plants, meteorites, xeroderma pigmentosum, animal husbandry and more.
This paper, authored by a second-year kindergarten teacher, explores her visit with a Navajo mother and her two children, both of whom have Xeroderma Pigmentosum (XP), a light sensitive condition often associated with mental retardation, beating loss, and developmental disabilities.
He is challenging other drivers to take up the cause of the Xeroderma Pigmentosum Society, a not-for-profit organization that raises money and awareness for children who cannot be exposed to light, natural or artificial.
There's a nasty confrontation, involving a gun, then Oliver explains that he has a rare skin disorder, called xeroderma pigmentosum, which vampires had used to incubate a vampire infection that could be passed on by close contact.
lymphoma), radiotherapy, scarring, xeroderma pigmentosum, or basal cell nevus syndrome.

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