Epidermodysplasia Verruciformis

What is Epidermodysplasia Verruciformis?

Epidermodysplasia Verruciformis is an inherited skin condition. It is characterized by wart-like lesions on the skin. These lesions may turn into skin cancer and this is aided through exposure to ultraviolet radiation. This condition makes you more prone to infections that are caused by human papillomavirus (HPV) ⁴.

Epidermodysplasia Verruciformis manifests mostly in childhood but there are reported cases of individuals who are affected at infancy and in puberty. This condition affects both men and women equally.

Causes

Genetic factors

Epidermodysplasia Verruciformis can result from mutations in the EVER1 and EVER 2 genes. These genes are responsible for coding membrane proteins that work together with keratinocytes, a type of epidermal cell which manufactures keratin. When mutation occurs in these genes, keratinocytes become more prone to human papillomavirus infections.

Mutation also occurs in other genes such as LCK, MST1 and RHOH which causes abnormalities in T cell receptors and T-lymphocytes. T cells are vital in immune responses and when mutation occurs in these cells, your immune response is diminished which makes you vulnerable to human papillomavirus infections. These defective genes are passed from an affected parent to a child.^1,2

Risk factors

There a number of factors that can elevate your chances of developing this condition. They include:

• When you come from a family where this condition exists, you are likely to develop it.
• Having HIV can make you more vulnerable to epidermodysplasia verruciformis.
• Those using immunosuppressive drugs since they had undergone an organ transplant are at risk of developing this condition.
• When you have cancer such as lymphoma, you are likely to develop epidermodysplasia verruciformis.⁴

Clinical Presentation

Symptoms of this condition start when you are a child and progresses as you grow. The skin lesion may not change after developing or disappear and recur after healing. The following are symptoms of this condition:

People have noticeable flat lesion or raised skin lesion. The flat lesion may be solid papules which are about I mm in diameter and are scaly. They may be pink, reddish- brownish form a plague on your skin as a result of some papules coming together. Raised skin lesion may appear oily, scaly, thick and brownish.

These lesions can appear on several parts of your body such as:

• Chest
• Back
• Abdomen
• Hands
• Soles
• Palms
• Feet
• Arms
• Legs
• Outside genitals
• Head
• Neck
• Chin
• Earlobes

In some cases, these lesions may appear as small numbers on your body and sometimes many of them group together on your body to form a big bunch. When this occurs, it forms dislocated, uneven rough bark like look on the skin thus giving this condition another name called Tree Man Syndrome.

Diagnosis

Several methods can be used to diagnose epidermodysplasia verruciformis such as:

• Physical exam: Your doctor assesses your family past health history and symptoms to get information to help in diagnosis of this condition.
• Skin biopsy: Your doctor extracts the skin on affected area and takes it for pathological examination.
• Immunohistochemical analysis: This is a method used to determine the presence and location of protein in tissues. Your doctor conducts this analysis by using a certain antibody that is bind in the skin sample and observes it under a microscope. The information obtained through this analysis helps your doctor to assess and treat epidermodysplasia verruciformis.

Your doctor should also conduct other tests to exclude other similar conditions such as carcinoma.²

Management and Treatment

Epidermodysplasia verruciformis can be treated using the following methods:

Tropical retinoids

These are types of medicines obtained from Vitamins A. They are used to improve intercellular communication by allowing surrounding cells to send signals to each other and control the growth and production rate of epithelium cells. Tropical retinoids have long been used to treat several medical conditions such as cancer and skin conditions like acne, skin aging and psoriasis. Examples of tropical retinoids include tretinoin, alitretinoin, tazarotene and adapalene. These medications are available as gel, cream or liquid. You have to apply them on the affected skin once per day after you have cleaned the affected area. After cleaning, wait for about 30 minutes before applying these medications.⁷

When tropical retinoids is used for a long time and in large doses, it causes side effects such as:

• It makes your skin to be red and scaly.
• It also causes your skin to be dry and itchy.

Surgery

Several surgery procedures can be used to treat epidermodysplasia verruciformis. They include:

Laser ablation

This is a technique that uses a beam of light to destroy unwanted tissues on the body.

Electrosurgery

This method involves using electricity to heat and eliminate unwanted tissues by vaporizing, dehydrating and coagulating them. There are two forms of electrosurgery that can be used: electrocautery and high frequency electrosurgery. In high frequency electrosurgery, your doctor uses high frequency alternating current which is changed into heat as it goes through the affected area in your body. The heat destroys unwanted cells or tissues on your body. In electrocautery, direct heat is used to destroy the abnormal tissue and cells. Your doctor uses electric current to heat a particular hand held device which is then applied on the affected area on your body.⁵

Cryosurgery

It is also known as cryotherapy, it involves using very cold temperatures to eliminate cancerous cells. In this method, your doctor applies liquid nitrogen or argon directly on the affected area on your body with a spraying device. This freezes the abnormal tissue and helps relieve symptoms.⁶

Prevention

Currently, studies have not yet found methods to effectively prevent this condition. However, if this condition runs in the family, you need genetic counseling to assess the risk before deciding to get a child.

This condition worsens when you are exposed to ultraviolet radiation. Therefore, avoid direct sunlight and protect yourself every time you are in the open. In addition, go for regular health checkup to help you detect any abnormal lesion on your body and manage it effectively in its early stages.¹

Images

Reference List

1. http://www.dovemed.com/diseases-conditions/epidermodysplasia-verruciformis/
2. Epidermodysplasia Verruciformis http://www.healthline.com/health/epidermodysplasia-verruciformis#pictures3
3. Epidermodysplasia Verruciformis http://www.mdedge.com/cutis/article/101957/epidermodysplasia-verruciformis-successful-treatment-squaric-acid-dibutylester
4. Epidermodysplasia Verruciformis https://www.dermnetnz.org/topics/epidermodysplasia-verruciformis/
5. Electrosurgery http://emedicine.medscape.com/article/1997619
6. Cryosurgery https://www.cancer.gov/about-cancer/treatment/types/surgery/cryosurgery-fact-sheet
7. Tropical retinoids http://www.aocd.org/?page=Retinoidstopical