Definition

What is Epidermodysplasia Verruciformis?

Epidermodysplasia verruciformis (EV) is an extremely rare skin disease that occurs when wartlike lesions cover parts of the body. It’s an inherited condition that makes a person highly susceptible to infections caused by the human papillomavirus (HPV).

How common is Epidermodysplasia Verruciformis?

The exact prevalence of Epidermodysplasia Verruciformis is not known. Till date, over 200 cases have been reported in the medical literature. EV can affect both male and female genders and no preference is noted. Epidermodysplasia Verruciformis occurs without racial or ethnic predilection. The manifestation of EV typically occurs in childhood; although, there have been cases of individuals being affected in infancy and puberty as well. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Epidermodysplasia Verruciformis?

The common symptoms of Epidermodysplasia Verruciformis are:

  • Flat-topped or bumpy lesions
  • Small, raised bumps known as papules
  • Large patches of raised and inflamed skin, known as plaques
  • Small, raised brown lesions that resemble scabs

Flat lesions are more common in areas exposed to sunlight, such as:

  • Hands
  • Feet
  • Face
  • Ears

Many of these lesions take on the appearance of tree bark or tree roots. Because of this, EV is sometimes referred to as “tree-man disease” or “tree-man syndrome.”

Growths may be limited to a small cluster of just a few warts or extend to more than 100 warts covering much of the body.

Plaques usually develop on the:

  • Neck
  • Arms
  • Armpits
  • Palms of the hands
  • Trunk
  • Legs
  • Soles of the feet
  • External genitals

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Epidermodysplasia Verruciformis?

EV is a type of genetic disease known as an autosomal recessive inherited disorder. That means a person must have two abnormal EV genes — one from each parent — to develop EV. In most cases of EV, the genetic mutation is sporadic, meaning it developed when the sperm or egg first formed. These genes can still be passed on to future offspring.

About 10 percent of people with EV have parents who were blood relatives, meaning they shared a common ancestor.

People who have EV have a normal immune response to non-HPV infections. However, for reasons still not completely known, these individuals are more prone to infection with certain HPV subtypes. There are over 70 HPV subtypes that can cause warts. But the subtypes most often found in EV are not the same as the those that most commonly cause genital warts and cervical cancer.

People with EV may have a poor immune response to HPV or other wart viruses. There have been about 30 HPV subtypes identified as triggers for warts and plaques in people with EV.

Although symptoms primarily appear by puberty, EV can develop at any age. Males and females appear to be equally at risk.

Risk factors

What increases my risk for Epidermodysplasia Verruciformis?

There are many risk factors for Epidermodysplasia Verruciformis, such as:

  • A family history of the disorder
  • Being born to parents who are close blood relatives (such as first cousins); about 10% of the reported cases are known to be of children born in consanguineous marriages
  • Being infected with HIV
  • Having undergone an organ transplant, and therefore, being on immunosuppressive drugs
  • Being affected by cancers of the lymphatic system (lymphoma)

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Epidermodysplasia Verruciformis diagnosed?

The diagnosis of Epidermodysplasia Verruciformis is made using the help of the following tools:

  • Thorough physical examination and assessment of symptoms
  • Evaluation of family medical history
  • Biopsy of the lesion/affected skin, to check for dark patches (acanthosis), thickening of the outer skin layer (hyperkeratosis), and granular cells of the skin (hypergranulosis)
  • In-situ hybridization to check for HPV viral particles (using a probe to identify nucleic acid material of human papilloma virus in the skin sample)
  • Immunohistochemical analysis, in which an anti-HPV antibody is used to bind the virus in the sample, which is then visualized with a microscope
  • Tests, as appropriate to rule out other conditions that may have similar symptoms, such as squamous cell carcinoma

How is Epidermodysplasia Verruciformis treated?

There is no cure for EV, so treatment is primarily to alleviate symptoms. Although surgery to remove the lesions can be successful, it may only be a temporary solution. Lesions can develop again, though they may never come back or may take years to return.

One surgical option available is curettage. It involves the use of a spoon-shaped device called a curette. Your surgeon uses the curette to carefully scrape away a lesion. The goal is to preserve as much healthy skin under and around the lesion as possible.

EV warts may be treated as you would other types of viral warts. These treatments include:

  • Chemical treatments, such as liquid nitrogen
  • Topical ointments that contain salicylic acid
  • Cryotherapy, in which the wart is destroyed by freezing it

Another important aspect of treatment is limiting sun exposure and using sunscreen to help preserve the health of your skin. People with EV are at an increased risk of developing skin cancer. Following your dermatologist’s advice about skin care and sun protection is very important. In fact, up to two-thirds of people with EV will develop skin cancer, usually in their 20s or 30s.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Epidermodysplasia Verruciformis?

The following lifestyles and home remedies might help you cope with Epidermodysplasia Verruciformis:

  • Use sun block or sun protection.
  • Maintain good hygiene.
  • Adopt a balanced diet with fresh fruits and vegetables.

If you have any questions, please consult with your doctor to better understand the best solution for you.

 

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: August 17, 2018 | Last Modified: August 17, 2018

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