What’s epidermodysplasia verruciformis?

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Epidermodysplasia verruciformis is a genetic disorder that can cause outbreaks of flat warts throughout the body and increase a person’s chance of developing skin cancer. People with the disorder develop physical symptoms only if they come into contact with human papillomavirus (HPV), a common sexually transmitted disease that can also be passed on from the mother during childbirth. Patients with epidermodysplasia verruciformis typically need to avoid sun exposure, use prescribed topical creams, and attend frequent clinical wart removal surgeries to keep their symptoms to a minimum. There is no cure for the disease, and even with dedicated treatment, people suffer from lifelong skin complications.

There are several dozen known strands of HPV, and most cause no 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 to proliferate through the outer layers of the skin. Warts usually first appear in infancy or early childhood in patients with congenital HPV.

Epidermodysplasia verruciformis warts can appear anywhere on the body, but are often more 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 don’t itch, cause pain, or cause adverse health consequences. It is possible for some warts to become cancerous, especially in the elderly and in patients who are not extremely careful about avoiding direct sunlight. Malignant lesions can be identical to benign flat warts or become hard, raised, brown masses on the skin.

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

Treatment for epidermodysplasia verruciformis involves a multifaceted approach to reducing the risk of cancer and improving the patient’s physical appearance. Because ultraviolet light appears to worsen the condition, it is important for patients to wear protective clothing and use sunscreen whenever they venture outdoors. Oral medications and topical retinoid creams, such as imiquimod, are often used to slow the growth of abnormal skin cells. Surgical approaches to treatment include freezing the warts with liquid nitrogen, burning them with electric heating devices, and excising them with a scalpel. The 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 surrounding skin and replace it with a graft from an unaffected area of ​​the patient’s body. If the cancer spreads to lymph nodes or other organs, a person may also need to have chemotherapy and radiation therapy.

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