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What is an Epidermodysplasia Verruciformis?

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  • Written By: D. Jeffress
  • Edited By: Jenn Walker
  • Last Modified Date: 06 December 2016
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Epidermodysplasia verruciformis is a genetic disease that can cause body-wide outbreaks of flat warts and raise a person's chances of developing skin cancer. People with the disorder only develop physical symptoms if they come into contact with human papillomavirus (HPV), a common sexually transmitted disease that can also be passed down from a mother during childbirth. Epidermodysplasia verruciformis patients typically need to avoid sun exposure, use prescription topical creams, and attend frequent clinical wart-removal surgeries to keep their symptoms at a minimum. There is no cure for the disease, and even with dedicated treatment, people suffer from skin complications their entire lives.

There are several dozen known strands of HPV and most do not cause problems in relatively healthy people. An individual with epidermodysplasia verruciformis, however, is highly sensitive to many different strands. A specific genetic mutation impairs the body's ability to control HPV infection and the virus is able proliferate throughout the outer layers of skin. Warts usually first appear in infancy or early childhood in patients who have congenital HPV.

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Epidermodysplasia verruciformis warts can appear anywhere on the body, but they are often most highly concentrated on sun-exposed areas of skin, such as the hands, forearms, face, and neck. They are usually small, flat, and slightly pinker or redder than the surrounding flesh. While multiple lesions can be embarrassing, they typically do not itch, cause pain, or result in negative health consequences. It is possible for some warts to turn cancerous, especially in older adults and patients who are not extremely careful about avoiding direct sunlight. Malignant lesions may be identical to the benign flat warts or become hard, raised, brown masses on the skin.

Most cases of epidermodysplasia verruciformis are diagnosed well before the threat of cancer is present. Doctors can make a diagnosis by performing thorough physical exams and testing blood and skin samples for the presence of HPV. Genetic testing confirms that certain genes carry the characteristic mutations that cause epidermodysplasia verruciformis.

Treatment for epidermodysplasia verruciformis involves a multifaceted approach to reduce the risk of cancer and improve a patient's physical appearance. Since ultraviolet light appears to worsen the condition, it is important for patients to wear protective clothing and use sunscreen whenever they must venture outdoors. Oral drugs and topical retinoid creams, such as imiquimod, are often used to slow the growth of abnormal skin cells. Surgical approaches to treatment include freezing off warts with liquid nitrogen, burning them with electric heating devices, and excising them with scalpels. Lesions are very likely to return despite frequent treatments.

Patients who develop skin cancer often require additional procedures. A surgeon may decide to remove a cancerous wart and the surrounding skin, and replace it with a graft from a non-affected area of the patient's body. If cancer spreads to the lymph nodes or other organs, a person may need to undergo chemotherapy and radiation treatments as well.

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