2. What does mean scleroderma?
“Hardening of skin”
When a person has scleroderma, the body makes too much collagen. This
excess collagen, the substance that holds our body together, causes hardening
and tightening. (Hardening and tightening varies with type of scleroderma)
5. Classification
1. Systemic sclerosis
Diffuse cutaneous systemic sclerosis.
Limited cutaneous systemic sclerosis
Overlap syndrome
Localized scleroderma
Morphea
Linear scleroderma
En coup de sabre.
6. Diffuse cutaneous systemic sclerosis
Proximal skin thickening
Tendency to rapid progression of skin skin changes.
Rapid onset of disease following Raynaud’sRaynaud’s phenomenon.
Poor prognosis
7. Limited scleroderma
Raynaud‘s phenomenon for years, before onset of scleroderma.
Manifestation – calcinosis cutis, telangiectasia.
Distribution – restricted to acral parts and face.
Nail fold‘s - without capillary drop outs.
Pulmonary involvement- late and infrequent, as pulmonary hypertension.
8. Sets of sclerosis
Morphea on skin:- (red 🔴 or purple🟣) one or few patches of thickened skin. (It
affect only the outermost layers of skin-dermis and fatty tissue.
1. Patches can itch
2. Painless
3. sometimes the excess collagen develops deep in the skin.. In rare cases affects
muscle
4. Location-abdomen, chest and back (face, arms and legs)
9. Patches of morphea on patients back
Generalization morphea
Patches of morphea can develop on different locations of the body.
Patches can grow together.
Band of thickening skin on arm or leg.
Can go deep into the tissue beneath the skin.
10. Linear morphea on lower leg and foot
Linear scleroderma
Often beginnings in childhood or the teenager’s year.
Thickening of skin(arm or leg).
Hardening can extend beyond the skin, going deep into the muscle, sometimes
the bone.
11. En coup de sabre on forehead
Location:- scalp, face or both.
Person with mild case develops a bit of tissue loss.
And tissue beneath disappears.
Such as hyaluric acid to restore the face.
12. Manifestation
• Hard, thickening or tight skin.
• Hair loss and less sweating.
• Dry skin and itch.
• Skin color changes
• Salt and pepper look to the skin.
• Stiff joints and difficult moving
them.
• Muscle shortening and
weakness.
• Loss of tissue beneath the skin.
• Bone may not grow as it should.
• Sores and pitted scars on the
fingers.
13. How to diagnosed it?
Dermatologist and rheumatologist.
1. Symptoms /signs > medical history
2. Skin biopsy
Other tests blood tests,
Why do x ray?
MRI
Scleroderma
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17. Is it treatment helpful, if starts early?
Physical and occupational therapy.
Phototherapy and medicine like methotrexate (banned in pregnancy) and
cyclosporine.
Important (how much deeper scleroderma is, type, what etiology).
18. Swelling and patches of hard feeling skin – calcipotriene (+strong corticosteroid)
Dry and Itching – moisturizer, camphor, menthol.
Calcium deposits beneath the skin – soaking in warm water, prednisolone, laser
treatment.
Morphea on top layers of the skin – narrow band UVB – 1 phototherapy.
(Before it take medicine called psoralen).
Visible blood vessels - laser therapy.
Hardness reaches behind your skin into tissue below – methotrxate , corticosteroid
by mouth.
Extracorporeal photopheresis (ECP) – severe scleroderma that affects skin and
internally.