Epidermodysplasia Verruciformis

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Transcript Epidermodysplasia Verruciformis


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,

This disease affects the keranocytes
located in the stratum basale layer and
the stratum corneum in the skin.

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 cellmediated immune response to HPV
infection. Many HPV types found in EV
are lesions and nonpathogenic to the
general population” (Kao, 2012).


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

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.

“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).

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.


Epidermodysplasia Verruciformis, is also
known by another name, LutzLewandowsky epidermodysplasia
verruciformis. After the first known case.



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
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