Inherited epidermodysplasia verruciformis

Inherited epidermodysplasia verruciformis (EV), sometimes called Lewandowsky-Lutz dysplasia, is a very rare genetic skin condition that makes a person's immune system unable to fight off certain strains of human papillomavirus (HPV). HPV is a common virus that most people's bodies control without any problem, but people with EV cannot clear it. As a result, the virus causes flat, wart-like growths and scaly patches to appear all over the skin, especially on the face, hands, arms, and trunk. These skin changes usually begin in childhood and continue throughout life. One of the most serious concerns with EV is that some of the HPV strains involved — particularly HPV-5 and HPV-8 — can cause skin cancers, especially a type called squamous cell carcinoma. These cancers tend to develop in areas exposed to sunlight, often in early adulthood. Because of this cancer risk, lifelong sun protection and regular skin checks are extremely important. There is currently no cure for EV. Treatment focuses on managing skin lesions, preventing new ones, and catching skin cancers early. Options include topical creams, retinoids (vitamin A-based medicines), and surgical removal of suspicious growths. Antiviral medicines have been tried but have limited success. People with EV need ongoing care from a team of specialists throughout their lives.

Bring these to your next appointment

• Q1.Which genes should be tested to confirm my diagnosis, and what does the result mean for my family members?,How often should I have my skin checked, and what warning signs of skin cancer should I watch for?,What sun protection measures are most important for me, and are there any specific products you recommend?,Are there any treatments — topical or oral — that could help reduce my skin lesions?,Should my parents, siblings, or children be tested for this condition?,Are there any clinical trials or new treatments I should know about?,Can you refer me to a genetic counselor to help me understand the inheritance and family planning options?

Common questions about Inherited epidermodysplasia verruciformis