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 Scleroderma  

 

 


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.

Blood tests may include:
*Antinuclear antibody (ANA) panel
*Antibody testing
*ESR (sed rate)
*Rheumatoid factor

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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