What is Scleroderma?

 

Scleroderma is an autoimmune disease affecting the skin and other organs of the body, meaning that the body’s immune system is causing inflammation and other abnormalities in these tissues. The main finding in scleroderma is thickening and tightening of the skin and inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas. There is still no cure for scleroderma, but effective treatments for some forms of the disease are available.

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What are the symptoms or features?

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Scleroderma differs from person to person but can be very serious.

Although symptoms vary greatly from patient to patient, it can dramatically impact someone’s life. Several other diseases that affect the skin are sometimes confused with scleroderma.

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The two main types of scleroderma are:

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1) Localized scleroderma. Localized scleroderma usually affects only the skin, although it can affect the muscles, joints and bones. It does not affect internal organs. Symptoms include discolored patches on the skin (a condition called morphea); or streaks or bands of thick, hard skin on the arms and legs (called linear scleroderma). When linear scleroderma occurs on the face and forehead, it is called en coup de sabre.

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2) Systemic scleroderma
Systemic scleroderma is the most serious form of the disease, can affect the skin, muscles, joints, blood vessels, lungs, kidneys, heart and other organs. There are two major forms of systemic scleroderma: limited cutaneous systemic sclerosis (formerly called CREST syndrome) and diffuse cutaneous systemic scleroderma.

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·        In Limited cutaneous systemic sclerosis (CREST syndrome), skin thickening and tightness is typically limited to the fingers and toes. The acronym stands for the formation of calcified nodules under the skin, Raynaud’s phenomenon, problems with movement of the esophagus, skin thickening and tightening of the fingers, and dilated blood vessels in the skin called telangiectasias.  A blood test called centromere antibodies are frequently seen in this form of scleroderma.

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·        In Diffuse cutaneous systemic scleroderma, skin thickening and tightness usually also extends from the hands to above the wrists. This form of scleroderma more frequently involves internal organs, such as lungs, kidneys, or the gastrointestinal tract. A number of newer antibodies are being discovered to classify this form of scleroderma, but the most common antibody is Scl-70 antibody.

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How is it diagnosed?

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Diagnosis can be tricky because symptoms may be similar to those of other diseases. There is not a single blood test that can say for sure that you have scleroderma, although numerous antibodies have been associated with this condition. To make a diagnosis, a doctor will ask about the patient’s medical history, do a physical exam and possibly order lab tests and X-rays. Careful clinical evaluation is the primary method for monitoring scleroderma. X-rays and computerized tomography (CT) scans are used to look for abnormalities. Thermography can detect differences in skin temperature between the lesion and normal tissue. Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment.

 

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How is it treated?

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While some treatments are effective in treating different aspects of this disease, there is no drug that has been clearly proven to completely reverse the key symptom of skin thickening and hardening however proper and aggressive treatment with advanced therapy can greatly improve prognosis and relieve some symptoms.