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.
Raynaud's Phenomenon Symptom
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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.
Scleroderma Research Foundation
Scleroderma Society of Canada
Kreuter A, Hyun J, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440-447.
Mathai SC, Girgis RE, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29:469-475.
Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38:219-223.
Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Accessed February 22, 2016.
Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.dynamed.com/topics/dmp~AN~T116347/Systemic-sclerosis. Updated June 9, 2016. Accessed September 13, 2016.
What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients_whatis.html. Accessed February 22, 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 February 2016 by Michael Woods, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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