Epidermodysplasia Verruciformis

Kyra Barrett and Darian Hoaks

The Epidemiology

EV is an uncommon, rare, either inherited disorder or contracted infection through the Human Papilloma Virus (HPV). Where depending on your severity you could suffer from wart-like lesions or acquire skin so rough it begins to look like tree bark.

Wart-like lesions on the face,Tree trunk –

like skin surrounding the hands

What area of the Integumentary System are affected?

This disease affects the keranocytes located in the stratum basale layer and the stratum corneum in the skin.

How is it affecting the Integumentary System ?

Depending on how you come in contact with EV, environmentally or inherited, you suffer from an increased growth rate of the stratum corneum which increases the thickening process of the skin and causes the “tree-trunk” appearance.

In EV, if you contract it through HPV, it mainly affects your “EVER1 and EVER2 genes, the cause unknown, one hypothesis states that they are involved with keratinocytes, or fail to respond to the initial immune response itself”.

“EV variants such as immunosupression: HIV infection, organ transplant, or idiomatic lymphopenia.”

“Patients with EV have a defective cell-mediated immune response to HPV infection. Many HPV types found in EV are lesions and nonpathogenic to the general population” (Kao, 2012).

How Do You Get EV?

You can get EV one of two ways: inheritance and environmental factors, such as HPV.

Inheritance

Patients who acquire EV through inheritance have it in their family gene pool through incest. This is more commonly known as inbreeding.

When inbreeding occurs the percent of autosomal or sex-linked recessive diseases or disorders increase its appearance in the next generation. › In genetic calculations this is where the “inbreed

coefficent” increases the likelihood of the recessive disease to show in the next generation.

Parent Generation

A

A

A

a

AA

AA

Aa

Aa

Recessive Trait

Situation: The parents here have two children. A boy and a girl. Each carry the recessive trait for EV. They marry and have children. Here are their genes.

F2

f2

F1 f1

FF

Ff

Ff

ff

This genotypic cross is increased by the inbreeding coefficient which then increases your chances of having the disease by more than 25%

(This is just one possible scenario.)

With F1 and F2 being the offspring of A1 and A2 the genotypic cross shows the possible ratios of EV in this family.

Environmental

The main environmental cause of EV is immune deficiency by the disease HPV. Or a reduced immune system through HIV, which can be contracted through blood-to-blood or bodily fluid contact.

Age and Target Group

“EV is universal and affects persons of all races” and “there is no sexual preferences noted for EV” and finally “patients with EV signs are typically present in early childhood with flat wart-like lesions on the extremities and face” (Kao, 2012).

Prognosis

Individuals who have EV often also develop frequent malignant skin tumors and other cancers. So, after developing said tumors they go through regular chemotherapy and radiation treatments with their rate of survival depending on types of cancer they develop.

Treatments and Research

As mentioned in the previous slide regular chemotherapy and radiation is often given as a treatment.

Because so little about EV doctors are often looking for the actual reasoning on how you can develop and make the daily lives of the patients more enjoyable.

Interesting Facts

Epidermodysplasia Verruciformis, is also known by another name, Lutz-Lewandowsky epidermodysplasia verruciformis. After the first known case.

Pictures

References

Epidermodysplasia verruciformis. DermNet NZ. (n.d.). DermNet NZ. Facts about skin from New Zealand Dermatological Society.. Retrieved December 14, 2012, from http://www.dermnetnz.org/viral/epidermodysplasia-verruciformis.html

Kao, G. F. (2012, January 26). Medscape: Medscape Access. Medscape: Medscape Access. Retrieved December 14, 2012, from http://emedicine.medscape.com/article/1131981-overview

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