Last reviewed by Dr. Raj MD on January 12th, 2022.
What is Epidermodysplasia verruciformis?
It is an autosomal recessive genetic skin disorder with a classic manifestation of wart-like lesions that grow in various parts of the body. It is caused by the human papillomavirus (HPV).
People with epidermodysplasia verruciformis have an impaired immune response to human papillomavirus. The skin lesions of epidermodysplasia verruciformis could develop into skin cancers, especially when exposed to the extreme heat of the sun for a long period of time. (1, 2, 3)
Epidermodysplasia verruciformis Pictures
Image 1: A classic manifestation of a person with epidermodysplasia verruciformis.
Photo Source: image.slidesharecdn.com
Picture 2: A person with a mild case of epidermodysplasia verruciformis.
Image Source: www.healthline.com
Who is prone to Epidermodysplasia verruciformis?
It is an autosomal recessive genetic disorder. In order for you to have the disease, you need the abnormal EV genes of both parents, thus, making the disease extremely rare. Although it is rare, epidermodysplasia verruciformis affects both men and women equally.
It also affects all races. Those who have a family history of Epidermodysplasia verruciformis are susceptible. People with HIV, cancer of the lymphatic system, taking immunosuppressive drugs, and have undergone a transplant are prone to Epidermodysplasia verruciformis. (3, 4)
Epidermodysplasia verruciformis is a genetic/inherited condition in which both parents have abnormal EV genes for the condition to be passed on to their children. Most cases of epidermodysplasia verruciformis are sporadic, during the sperm or egg formation, which can be passed on to offspring. (3, 4)
Epidermodysplasia verruciformis symptoms
Epidermodysplasia verruciformis symptoms can develop at any age, although it commonly appears by the time the patient reaches puberty. The classic manifestation of EV is the wart-like lesion that looks like a flat-topped papule characterized by its pinkish to violet color.
There are instances when the lesions clump together forming large plaques. It has scaly surface and irregular borders. Another distinct characteristic of EV lesion is the seborrheic keratosis-like lesion, which is common in sun-exposed skin.
The lesion is brown in color and slightly raised. This type of lesion is most likely to develop into cancer.
Tree-man disease/Tree-man syndrome
The lesions of EV resembles the roots and/or bark of the tree. That is why many people call it the Tree-man disease/tree-man syndrome. The patient’s body will be covered with a small cluster of warts. In severe cases, the entire body is covered with warts.
The commonly affected parts of the body are the arms, neck, trunk, palms of the hands, armpits, soles of the feet, legs, and even the external genitalia.
Clinical and histological procedures should be done to accurately diagnose the condition of the patient. The medical history, clinical manifestations, and laboratory results should also be considered.
A genetic testing registry (GTR) is done to find out the genetic components of EV. (4, 5, 6, 7)
A thorough examination of the epidermodysplasia verruciformis showed enlarged cells I the spinous layer of the skin with a distinct blue-grey inflamed cytoplasm. It is indicative of HPV infection, which is totally different than the typical type of HPV.
How to cure epidermodysplasia verruciformis? Sad as it may seem, but epidermodysplasia verruciformis is a lifelong disease. The lesions can be removed but it is just a temporary approach. Eventually, the lesions will come back. As a matter of fact, there are possibilities that the lesions will remain unchanged throughout the years.
About 50% of EV cases can progress into skin cancer in the forms of squamous cell carcinoma and basal cell carcinoma. The chances of malignancy are higher in people with EV who are between 30 years old and 50 years old.
While there is no existing cure or prevention for epidermodysplasia verruciformis, the best thing to do is to appropriately manage the condition of the patient. It is managed using a combination of medical and surgical remedy. Health education, counseling, and careful monitoring should also be a part of the management approach.
The following should be included in the management of epidermodysplasia verruciformis.
- It is important to stay away from the direct heat of the sun to avoid the possibility of turning epidermodysplasia verruciformis lesions into skin cancers.
- The doctor can also perform curettage in which a curette (spoon-shaped device) is used to scrape away the lesion. It is done to preserve the healthy skin.
- For warts, they can be removed using various strategies. Although, there is really no guarantee that the wart will not come back again. Some of the wart removal methods that can be used include:
- Chemical treatment using oral and topical retinoids, imiquimod, and fluorouracil.
- Laser ablation
- Cryotherapy using liquid nitrogen
- If the condition progressed to skin cancer, the best approach is surgical excision and reconstruction. (7, 8, 9)
- Epidermodysplasia verruciformis can result in severe emotional and psychological stress, especially if the lesions and warts are severe and covered the exposed parts of the body.
- There is a possibility of skin cancer in about 30% to 60% of patient with epidermodysplasia verruciformis. Malignancy commonly takes place in people between 30 years old to 50 years old.
- Metastasis rarely takes place. However, if the disease metastasized to other parts of the body, the condition can be extremely fatal.
Although there is no cure for epidermodysplasia verruciformis, it still has a fair prognosis as progression to skin cancer is less likely to happen, especially when exposed to sunlight is avoided. Metastases is extremely rare too. (1, 5, 9, 10)
Key points to remember
- Epidermodysplasia verruciformis is also known by the name Tree man syndrome, tree man disease, and Lewandowsky-Lutz syndrome.
- Epidermodysplasia verruciformis is a rare skin disease with the classic manifestation of wart-like lesions caused by human papillomavirus secondary to a weak immune system and genetic mutation.
- Warts appear in a small group or in clusters, which looks like a bark of a tree.
- Epidermodysplasia verruciformis is both an inherited and acquired disease.
- There is no cure for Epidermodysplasia verruciformis but the lesions can be surgically removed. Although, the recurrence rate is high.
- Epidermodysplasia verruciformis affects both men and women, age, and ethnicity. (2, 5, 9, 10)