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There is no cure for xeroderma pigmentosum (XP) and the skin deterioration that occurs with the disease is cumulative and often irreversible. People with this condition often require total, lifelong protection from sunlight or ultraviolet (UV) radiation-emitting agents (e.g., mercury-vapor lamps, tanning booths).
Once XP is diagnosed, each aspect of the disease may be treated and monitored by a different specialist. A dermatologist specializes in skin conditions and may treat the photosensitivity and skin cancer. An ophthalmologist specializes in eye diseases and may treat conjunctivitis (inflammation of the eyelid and lining) and eye tumors or growths. A neurologist specializes in diseases that affect the nervous system may screen for (and treat) any neurological complications (e.g., learning disabilities).
Treatment of skin cancer, a common complication of XP, centers on the prompt removal of any precancerous and cancerous tissue. For most squamous cell and basal cell carcinomas, which rarely spread, a biopsy is often the only necessary treatment. However, some carcinomas and melanomas, the most severe form of skin cancer, require other forms of cancer treatment. These may include radiation (therapy that uses high-energy rays, such as x-rays, to kill or shrink cancer cells), chemotherapy (drug therapy for inhibiting or destroying cancer cells) and specific skin techniques including:
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Cryosurgery. Freezing the affected area with liquid nitrogen.
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Curettage and electrodesiccation. Lesion is removed and any remaining layers of cancer cells are scraped away using a curette (circular blade).
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Surgical excision. Surgical removal of the suspicious lesion and some surrounding healthy tissue.
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Laser therapy. Narrow beam of intense light that vaporizes growths.
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Mohs surgery. The lesion is removed one layer at a time and examined under a microscope. The process is repeated until only healthy tissue remains.
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Skin grafting and reconstructive surgery. Healthy skin is moved from another area, such as the buttocks or thighs, and replaces cancerous tissue.
For the treatment of eye-related complications in patients with XP, an ophthalmologist may recommend the following:
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Surgery. The surgical removal of any noncancerous and cancerous tumors or growths in the eyes or eyelids.
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Artificial tears and/or soft contact lenses. The regular use of artificial tear drops and contact lenses may help soothe abnormally dry or irritated eyes in XP patients.
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Corneal transplant. If the eyes of an XP patient become so clouded that he or she cannot see, a corneal transplant may be considered to restore vision.
Sometimes patients with XP may develop neurological complications including progressive hearing loss, which may lead to deafness, cognitive disabilities and the progressive loss of nerve and muscular functioning. Although nothing can prevent or stop these problems from occurring, early screening and treatment for potential neurological complications may help patients to better cope with these. For instance, early detection of hearing loss and subsequent use of a hearing aid may lessen difficulties in communication for patients with XP. In addition, XP patients and their families face many daily challenges due to the disease’s various long-term physical, emotional, social and economic consequences. Therefore, patients with XP and their families may benefit from psychological counseling and support groups. |