Xeroderma Pigmentosum Essay

Xeroderma Pigmentosum

October 27, 2012 In 1874 Hebra and Kaposi were the first ones to describe the condition of Xeroderma Pigmentosum. It is a rare autosmoal recessive disorder, which is a form of cell that loses its ability to repair DNA. The sun can affect every part of the epidermis, which is the top layer of your skin. It makes your skin sensitive to the ultraviolet rays. The loss of un- repaired DNA builds up and causes cancerous cells. That is why when a patient is diagnosed with XP, they can’t be expose to the sunlight because of the ultraviolet rays. Usually the patients family history plays a role in this condition, once they have been diagnosed with Xeroderma pigmentosum. Xeroderma Pigmentosum is a genetic disease, the skin is sensitive to the ultraviolet rays and can cause skin cancer if exposed to sunlight. When a baby is born with XP, usually their skin appears to be normal at birth. Once the baby turns 6 months after birth, the first stage of XP starts to occur. The symptoms of a 6 month old baby will start developing freckles and when the baby is exposed to the sun the baby’s face will show a reddening on the skin. The reddening of the skin can also progress in their neck, lower legs, and in severe cases the trunk (abdominal) may be involved. Also the 6 month old baby will develop in their skin irregular dark spots. If the baby is exposed to the sunlight frequently it will lead he or she to the second stage of XP symptom. In the second stage the characteristic of pikiloderma will progress in the skin. The symptom of pikiloderma are when a spider web-like collection of blood spots and vessels are seen through the skin. The third stage of XP, occur at a early age 4-5 years old. In the third stage the symptoms that develop are solar keratoses and skin cancer. Solar keratoses is a abnormal skin cell that develop, due to ultraviolet radiation. They are considered to be precancerous. Skin cancer occur more often in people that are diagnosed with Xeroderma Pigmentosum. Another problem that occurs in the 3rd stage is in the eyes. Nearly 80% of XP patients eyes become painfully sensitive to the sun (photophobia). When eyes are exposed to the sunlight they become irritated, bloodshot and clouded. Also 20% of the patients with XP have neurological problems. The nerve- related problems are poor coordination, spastic muscle, and developmental delay. Once the neurological has occurred they keep on worsen overtime. Unfortunately there hasn’t been any cure yet for Xeroderma Pigmentosum. The main goal for those patients that have been diagnosed with XP should keep there skin and eyes protected from the ultraviolet lights. These are ways that can help with the symptoms of XP. When a patient with XP are outdoors in daylight, they must wear clothing that are tightly woven fabric so light wont go through. For example they must wear long sleeves and pants, shirts with collars, hats that are wide brimmed. To protect their eyes they should wear sun glasses that block ultraviolet lights completely. The patient must also put on sunscreen that contains chemical zinc, numerous oxides and kaolin. The sunscreen must be worn daily and has to be applied to all exposed areas of the skin. Those patients affected with Xeroderma Pigmentosum should be examined by a dermatologist every 3 to 6 months. Any suspicious spot or growth must be reported to the patient dermatologist. The dermatologist will detect the skin cancer before they have grown. If the dermatologist finds a small piece of suspicious skin growth he or she will remove the piece and will be examined for cancer. Frequent eye examination should also be done by a ophthalmologist. The eye doctor will help detect for eye cancer or any other problems that can occur in the eye. There hasn’t been any treatment for the symptom of neurological problems. While nothing can stop these problems from

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