Epidermodysplasia Verruciformis

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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.

Tree trunk – like skin surrounding the hands

Wart-like lesions on the face,

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

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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.

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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 cellmediated immune response to HPV infection. Many HPV types found in EV are lesions and nonpathogenic to the general population” (Kao, 2012). 

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

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.

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.

Parent Generation A

a

A AA

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

Aa Recessive Trait

A

AA

Aa

(This is just one possible scenario.)

F1 F2 This genotypic cross is increased by the inbreeding coefficient which then increases your chances of having the disease by more than 25%

f2

FF

Ff

f1 Ff

ff

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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.

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“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 wartlike lesions on the extremities and face” (Kao, 2012).

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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.

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. 

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

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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/epidermodysplasiaverruciformis.html Kao, G. F. (2012, January 26). Medscape: Medscape Access. Medscape: Medscape Access. Retrieved December 14, 2012, from http://emedicine.medscape.com/article/1131981overview APA formatting by BibMe.org.