Scleroderma means "hard skin". It is an autoimmune disease causing
inflammation and thickening in the skin and elsewhere, producing areas of
tight, hard skin. It may affect one area of the body or many systems.
| Type | What it affects |
|---|---|
| Localized scleroderma | Only the skin, and the muscles and tissue just beneath it |
| Systemic scleroderma (systemic sclerosis) | More serious — many parts of the body, and it can damage blood vessels and internal organs including the heart, lungs and kidneys |
The mechanism, and what causes it
The exact cause is unknown. Several factors appear to contribute:
- Genetics. Certain genes raise the chance of developing it and may
influence which type. Scleroderma is not passed from parent to child,
but you are more likely to develop it if a close relative has it. - Environment. Exposure to viruses or chemicals may trigger it.
- Immune system changes. When the immune system changes, it can trigger
cells to make too much collagen — and too much collagen is what produces
patches of tight, hard skin. That is the disease in one sentence. - Hormones. Hormonal or immune differences between women and men may play
a part.
Anyone can get it. It is more common in women, usually appears **between
30 and 50**, and can affect African Americans more severely.
Symptoms, by type
Localized scleroderma makes patches of thick, hard skin in one of two
patterns:
- Morphea — firm oval patches that stay in one area or spread to other
areas of skin - Linear scleroderma — lines of thickened or differently coloured skin
running down an arm or leg, and rarely on the forehead
Systemic scleroderma affects internal organs as well as skin: thick tight
skin on the fingers, fatigue, Raynaud's phenomenon (narrowing of the blood
vessels in the hands or feet), and damage to the digestive system, lungs,
kidneys and heart.
Diagnosis
There is no single test. Symptoms vary between people and resemble other
diseases, which makes it hard to diagnose. A provider will ask about symptoms
and medical history, do a physical exam, may order blood tests including an
ANA (antinuclear antibody) test, may do a skin biopsy, and may use
imaging to check for organ damage.
Treatment, including the part people do not expect
There is no cure, and treatment controls symptoms and limits damage:
- Medicines to reduce swelling, manage pain, control other symptoms and
prevent complications - Physical or occupational therapy for pain, muscle strength, and ways to
manage daily living - Regular dental care — because scleroderma dries the mouth and damages
the connective tissue in it, which **speeds up tooth decay and can loosen
teeth**
That last one belongs on the list as much as the first two, and it is the one
most likely to be left off a treatment plan.
Most people with scleroderma see a rheumatologist, a doctor specialising
in rheumatic diseases. Dermatologists often play an important role too, and
organ damage brings in further specialists.
What you can do yourself
- Dress warm and avoid cold or wet environments
- Quit smoking
- Sunscreen before going outdoors
- Moisturisers to lessen stiffness
- Avoid hot baths and showers, harsh soaps and household cleaners
- Regular physical activity
*Source: National Institute of Arthritis and Musculoskeletal and Skin
Diseases, via MedlinePlus.*
ライセンス: CC0 1.0(パブリックドメイン) · 出典 medlineplus.gov
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