2. Systemic Sclerosis
Background:
Systemic sclerosis is a systemic connective tissue
disease. Characteristics of systemic sclerosis include
essential vasomotor disturbances; fibrosis; subsequent
atrophy of the skin, subcutaneous tissue, muscles, and
internal organs (e.g. alimentary tract, lungs, heart, kidney,
CNS); and immunologic disturbances accompany these
findings.
3. Systemic Sclerosis
Epidemiology:
●estimated to occur in 2.3-10 people per 1 million.
●female-to-male ratio of 3-6:1
●usually appears in women aged 30-40 years, and it
occurs in slightly older men.
Types of Systemic sclerosis:
●Localized Scleroderma:
They affect only the skin (and sometimes the underlying
muscles and bones) but do not affect the internal organs, or
reduce life expectancy
●Systemic Scleroderma:
The systemic forms can affect any part of the body (skin, blood
vessels, and internal organs.
4. Systemic Sclerosis
Types of Systemic sclerosis:
A)Localized Scleroderma:
They affect only the skin (and sometimes the underlying muscles and
bones) but do not affect the internal organs, or reduce life expectancy
1-linear scleroderma:
is a line of thickened skin which can affect the bone and muscles
underneath it , thus limiting the motion of the affected joints and
muscles. It most often occurs in the arms , legs , or forehead, and may
occur in more than one area .
2-Morphea scleroderma:
usually begins as patches of discolored, dry skin m there then become
hard , slightly depressed , oval shaped plaques whitish usually have a
whitish or yellowish center surrounded by a pinkish or purplish halo
5. Systemic Sclerosis
Types of Systemic sclerosis:
B)Systemic Scleroderma:
The systemic forms can affect any part of the body (skin,
blood vessels, and internal organs.
6. Systemic Sclerosis
Types of Systemic sclerosis:
B)Systemic Scleroderma:
The systemic forms can affect any part of the body (skin,
blood vessels, and internal organs.
7. Systemic Sclerosis
:Histopathology
Intima proliferation
in a deep arteriole.
(A) Prominent inflammatory infiltrate particularly in the perivascular region is
observed in the early stage of diffuse cutaneous systemic sclerosis.
(B) In the later stage of the disease, there is increased extracellular matrix
deposition. Note the loss of dermal papillary structures.
10. Cutaneous Manifestation :
A)Raynaud phenomenon:
Recurrent
vasospasm white (pallor) blue (cyanosis) red (hyperemia).
-usually (but not always) precedes skin changes by several months
- usually occurs in response to stress or cold exposure
- Symmetrical acral.
- Affected areas also show at least two color changes
12. Cutaneous Manifestation :
B)Skin thickening:
3- Atrophic:
thin and atrophic
after many years .
1- Edematous :
swollen for weeks or months
2- Indurative :
thickened and tightly adherent
Phases:
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
13. Cutaneous Manifestation :
-elongated face
-tight shiny skin
-loss of skin wrinkles and facial
expression
-Loss of nasolabial fold
-pinched nose
-causing pursed lips(Radical
furrowing)
-small oral opening (difficulty in
chewing and oral hygiene)
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: Face :
14. Cutaneous Manifestation :
-elongated face
-tight shiny skin
-loss of skin wrinkles and facial
expression
-Loss of nasolabial fold
-pinched nose
-causing pursed lips(Radical
furrowing)
-small oral opening (difficulty in
chewing and oral hygiene)
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: Face :
15. Cutaneous Manifestation :
-elongated face
-tight shiny skin
-loss of skin wrinkles and facial
expression
-Loss of nasolabial fold
-pinched nose
-causing pursed lips(Radical
furrowing)
-small oral opening (difficulty in
chewing and oral hygiene)
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: Face :
16. Cutaneous Manifestation :
-elongated face
-tight shiny skin
-loss of skin wrinkles and facial
expression
-Loss of nasolabial fold
-pinched nose
-causing pursed lips(Radical
furrowing)
-small oral opening (difficulty in
chewing and oral hygiene)
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: Face :
17. Cutaneous Manifestation :
tight skin over finger gradually
limits full extension and flexion
leading to contracture and skin
ulceration.
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: Sclerodactyly:
18. Cutaneous Manifestation :
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: localized
19. Cutaneous Manifestation :
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: localized
20. Cutaneous Manifestation :
1-phases
2-initial site and progression
3-skin appendages
4-Cutaneous pruritus is common.
5-Patterns :Diffuse :Face , Hand
localized :Linear , Morphea
B)Skin thickening: localized
32. • Management:
Treatment should be organ-based in order to try to
control the disease. Currently there is no cure. In
contrast to many other autoimmune diseases,
corticosteroids and immunosuppressants are rarely used
in SSc, with the exception of SSc-related pulmonary
fibrosis.
Systemic Sclerosis
