Scleroderma

By: AbdelAly Elbik 180223054

What is Scleroderma?

Terminology
• Prefix: Sclero = Hard
• Suffix: Derma = skin layer

Definition
It is considered to be described as a groub of rare diseases
combined together rather than skin hardening only but this
was appointed to be the most shown symptom during the
diagnosis as well as vascular and visceral abnormalities.
Scleroderma? Systamic Scelorosis?
The Pathophysiological Mechanism Of the Disease
How The Disease Effects The Body’s Functioning

Scleroderma is one of the most misunderstood, misdiagnosed disease due to its complexity
with other pathologies in terms of symptoms which effects its clear detection

• An Autoimmune disease where the the immune system start to

over-synthesize (products) collagen protein and extract it on the
skin making it more sclerostic & stiff.
Etiology
It cannot be transmitted from person to person
• Genatic make up: cannot be inherited, presence of HLA-complex, IRF5, STAT4.
• Hormons: womens are more vulnerable than men due to the change of
hormonal back up although findings on Estrogen involvement is not that much.
• History of assossiated diseases: the presence of other Autoimmune disease such
as Rheumatoid , RA and remarkably Systamic Lubus Arthematosis increases the
chance of developing Scleroderma.
“ The Disease varies in symptoms, effects, ways of
defect, severity, place and duration, in fact some
times the behavior of each findings do not relate
to eachother.

”
That’s why clinisions seprated them and made the
term scleroderma an umbrella for each.
Starting with

Localized Scleroderma
A localized Scleroderma patient would have a mild to
moderate problematic events and it will be mainly
superfasial.

Symptoms such as Morphea or linear are the main forms of it.

Morphea
• Thickened,red, oval
shaped patches on the
skin.
• Patches have little sweat
& hair.
• It could be a single patche
or mutable ones localized
or all over the body.
Linear
• A single line/band of skin
connective tissue lined stiffed
togther to form a hard line.
• Found mostly in legs and arms
and even in forehead
In here we will address the systemic events
assossiated with Scleroderma, this type is more
commen and it diffrs much more in symptoms and
variasions.
As we can understand from its name it’s spreaded
more, all over and deeper effecting mostly every
body organs & body’s systems mechanism (more like
a ZOMBIE).

Systamic Scleroderma
Systamic Scleroderma

Degrees of changes of the disease keeps changing

even in the zombie type for example there is the
limited Systamic Scleroderma , Sine systemic
Scleroderma, and lastly the Diffuse Systamic
Scleroderma and the severty of them is orderd.

Each has its own way of effectiveness on the body.

 Calciosis? raynaud’s?

Limited Scleroderma
Sclerodactyly?
 Limited Scleroderma
Limtied Scleroderma does not participate much in
terms of visceral involvement but still a systemic one.

What makes it different is that it targets the

superfasial areas wildly.

Its symptoms are controled in main 5 anomalies

gathered in one single word CREST
Calcinosis
It is a lumps of calcium
accumulate any where in
the body
After a while they can be
ruptured causing pain
It does not increases with
dite calcume intake
Raynaud’s Phenomenon
This changes on the
distal extremity
could be happened
due to whether
changes (cold) or
emotional stress
conctrecting the
blood vassels
Its important to keep in
mind this phenomenon
has stages.

The distal parts start to

be white (lack of blood),
then the color blue (lack
of oxygen) lastly red
(return of blood).
Esophageal Dysmotility
The esophagus becomes
less active and muscle
function decreases even
diminishes.

Over time muscles will be

replaced with scar tissues
resulting in diffeculety
swallowing, heart burn &
bloating.
Sclerodactyly
A localized thickening of
skin on fingers and toes.

The fingers while stay in

flexed position for long
and that will lead to
complecations such as
ulcers.
 Telangiectasia
A small red spots some times in
spider shaped appear on the
superfasial surface of the skin.

Happens due to damegs to large

and small blood vessels as well.

Found in: plams fingers, face,

lips & even tongue.
 In summary
• Limited scleroderma is the most common type
of Scleroderma.

• Its progression is accumulative, devolope very

slowly through over the years.

• Effecting the superfasial parts only not the

deeper ones although in some cases the internal
parts are effected but patially.
The Diffuse Scleroderma
This type of scleroderma can be the most sever one, it share the
same symptoms & signs with Limited one never the less it has
multi-internal involvements as well, the internal organs that can
be targeted is mostly the lung & kidney and other organs and
systems considerably.
Internal involvement starts as early as superfasial ones.
It targets 40% of all sclerodermatic pateints.
Problems in internal organs effect its function, mobility,
physiological motality and in some cases the total organ dysfuncion
and need to be distract and transplants with other in some lung
involvements for example
Sine Scleroderma

In this type fibrosis is only in

internal organs with out superfascial
appearance.

This is the most rare one of all