Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are 3 major forms of the disease:
Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.
Scleroderma is more common in women. The morphea type of scleroderma usually affects people 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.
Other factors that may increase your risk of developing scleroderma include:
Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with
Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. The fingers turn red after resolution of the symptoms. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. Other symptoms of diffuse scleroderma may include:
Complications of diffuse scleroderma can affect virtually every system of the body. Other common complications include:
You will be asked about your symptoms and medical history. A physical exam will be done. Diagnosis is based on changes in the skin.
Tests may include:
Imaging tests take pictures of internal body structures. These may include:
In addition, an esophagus motility study may be done
There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms in various systems.
Physical therapy and
to maintain circulation, joint flexibility, and
There are no current guidelines to prevent scleroderma.
Scleroderma Research Foundation
Scleroderma Society of Canada
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http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Updated August 2016. Accessed November 29, 2016.
Systemic sclerosis. EBSCO DynaMed website. Available at:
http://www.dynamed.com/topics/dmp~AN~T116347/Systemic-sclerosis. Updated June 9, 2016. Accessed November 29, 2016.
What is scleroderma? Scleroderma Foundation website. Available at:
Accessed November 29, 2016.
11/9/2015 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed: Kuo CF, Grainge MJ, Valdes AM, et al. Familial aggregation of systemic lupus erythematosus and coaggregation of autoimmune diseases in affected families. JAMA Intern Med. 2015;175(9):1518-1526.
Last reviewed March 2017 by
EBSCO Medical Review Board
Michael Woods, MD, FAAP
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