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Xeroderma pigmentosa is a rare condition passed down through families in which the skin and tissue covering the eye are extremely sensitive to ultraviolet light.
Causes, incidence, and risk factorsUltraviolet light -- such as that found in sunlight -- damages the genetic material (DNA) in skin cells. Normally, the body repairs this damage. But in persons with xeroderma pigmentosa, the body does not fix the damage. As a result, the skin gets very thin and patches of varying color (splotchy pigmentation) appear.
The condition also causes spidery blood vessels in the skin (telangiectasia) and skin cancer. Skin cancer often occurs before the child is 5 years old.
Symptoms- sunburn that does not heal after just a little bit of sun exposure
- Blisteringafter just a little bit of sun exposure
- Spider-like blood vessels under the skin
- Patches of discolored skin that get worse
- Crusting of the skin
- Scaling of the skin
- Oozing raw skin surface
- Discomfort when being in bright light (photophobia)
The doctor will perform a physical exam and ask if you have a family history of xeroderma pigmentosa.
An eye exam may show:
The following tests can help diagnose the condition in a baby before the birth:
- Amniocentesis
- Chorionic villous sampling
- Culture of amniotic cells
The following tests can help diagnose the disorder after the birth of the child:
- Culture of skin fibroblasts
- Skin biopsy
Children with this condition need total protection from sunlight. Even the light coming through windows and fluorescent bulbs is dangerous.
When these children must go out in the sun, they should wear protective clothing.
Wear high protection (SPF 70 or greater) sunscreen and very dark, UV-protected glasses. Your doctor may prescribe medicine to help prevent certain skin cancers.
Support GroupsXeroderma Pigmentosum Society -- www.xps.org
XP Family Support Group -- www.xpfamilysupport.org
Expectations (prognosis)Most persons with this condition die of skin cancer early in adulthood.
Complications- Change in appearance of skin (disfigurement)
- Skin cancer
Call for an appointment with your health care provider if you or your child has symptoms of xeroderma pigmentosa.
PreventionExperts recommend genetic counseling for persons with a family history of xeroderma pigmentosa who wish to have children.
ReferencesAbeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKena WG. Clinical Oncology. 3rd ed. Orlando, Fl: Churchill Livingstone; 2004.
Kumar V, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease. 7th ed. St. Louis, Mo: WB Saunders; 2005.
What else can I help you with?
Who discovered Xeroderma Pigmentosa?
hi everyone!!!!1 who discovered xero derma pigmentosa??????/?
Do people with xeroderma pigmentosa have DNA ligase?
Yes, people with xeroderma pigmentosa have DNA ligase. DNA ligase is an enzyme involved in the repair of DNA damage, which is particularly important for individuals with xeroderma pigmentosa who have a reduced ability to repair damage caused by ultraviolet (UV) radiation.
How do you use xeroderma in a sentence?
sentences of xeroderma
What is xeroderma?
The mildest form of acquired ichthyosis is called xeroderma, or dry flaky skin. It is not associated with any systemic diseases.
Does xeroderma mean dry skin?
Yes.
Is xeroderma pigmentosum disorder a genetic disease?
Yes it is.
What disease starts with the letter x?
· Xanthinuria · Xeroderma
Does Xeroderma means paper-thin skin?
yes
What is the history behind Xeroderma pigmentosum?
harry long weenie
When does xeroderma occur?
Xeroderma occurs most often on the lower legs of middle-aged and elderly adults during cold weather, or on the lower legs of people who bathe too often.
How many births does xeroderma pigmentosum occurs?
Approximately 1 in a million.
What is the medical term for scaly skin using the suffix derma?
xeroderma
