What is Scleroderma
cleroderma, or systemic sclerosis, is a chronic
connective tissue disease generally classified as one of the
autoimmune rheumatic diseases.
The word “scleroderma” comes from two Greek words: “sclero”
meaning hard, and “derma” meaning skin. Hardening of the skin is
one of the most visible manifestations of the disease. The disease
has been called “progressive systemic sclerosis,” but the use of
that term has been discouraged since it has been found that
scleroderma is not necessarily progressive. The disease may take
several forms which will be explained later. There is also much
variability among patients.
What
causes scleroderma?
The exact cause or causes of scleroderma are still unknown, but
scientists and medical investigators in a wide variety of fields
are working hard to make those determinations. It is known that
scleroderma involves overproduction of collagen.
what are the
symptom?
Some types of scleroderma affect only the skin, while others
affect the whole body.
*Localized scleroderma usually affects only the skin on the hands
and face. It develops slowly, and rarely, if ever, spreads
throughout the body or causes serious complications.
*Systemic scleroderma, or sclerosis, may affect large areas of
skin and organs such as the heart, lungs, or kidneys. There are
two main types of systemic scleroderma: Limited disease (CREST
syndrome) and diffuse disease.
Skin symptoms of scleroderma may include:
*Fingers or toes that turn blue or white in response to hot and
cold temperatures (See: Raynaud's phenomenon)
*Hair loss
*Skin hardness
*Skin that is abnormally dark or light
*Skin thickening, stiffness, and tightness of fingers, hands, and
forearm
*Small white lumps beneath the skin, sometimes oozing a white
substance that looks like toothpaste
*Sores (ulcers) on the fingertips or toes
*Tight and mask-like skin on the face Bone and muscle symptoms may include:
*Joint pain
*Numbness and pain in the feet
*Pain, stiffness, and swelling of fingers and joints
*Wrist pain Breathing problems may result from scarring in the lungs and
can include:
*Dry cough
*Shortness of breath
*Wheezing
*Digestive tract problems may include:
*Bloating after meals
*Constipation
*Diarrhea
*Difficulty swallowing
*Esophageal reflux or heartburn
*Problems controlling stools (fecal incontinence)
what are the Signs and tests to be done?
The health care provider will perform a physical exam. The exam
may show hard, tight, thick skin.
Your blood pressure will be checked. Scleroderma can cause severe
inflammation of small blood vessels, such as those in the kidneys.
Problems with your kidneys can lead to high blood pressure.
Other tests may include:
*Chest x-ray
*CT scan of the lungs
*Echocardiogram
*Urinalysis
*Tests to see how well your lungs and gastrointestinal (GI) tract
are working
*Skin biopsy
How serious is scleroderma?
Any chronic disease can be serious. The symptoms of scleroderma
vary greatly from individual to individual, and the effects of
scleroderma can range from very mild to life-threatening. The
seriousness will depend on what parts of the body are affected and
the extent to which they are affected. A mild case can become more
serious if not properly treated. Prompt and proper diagnosis and
treatment by qualified physicians may minimize the symptoms of
scleroderma and lessen the chance for irreversible damage.
What is the treatment for
scleroderma?
At the present time, there is no cure for scleroderma, but there
are many treatments available. Some are directed at particular
symptoms like heartburn, which can be controlled by medications
called proton pump inhibitors or medicine to improve the motion of
the bowel. Some treatments are directed at decreasing the activity
of the immune system. Some people with mild disease may not need
medication at all and occasionally people can go off treatment
when their scleroderma is no longer active. Because there is so
much variation from one person to another there is great variation
in the treatments prescribed.
what
are the Preventive measures?
There is no known prevention. Reducing your exposure to silica
dust and polyvinyl chloride may lower your risk for this disease.
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