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 three major forms of the disease:
Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma, but it is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production. There is also evidence that scleroderma may result from vascular abnormalities.
These factors increase your chance of developing scleroderma. Tell your doctor if you have any of these risk factors:
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 of fingers and toes. 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. Prominent complications include:
The doctor will ask about your symptoms and medical history, and perform a physical exam. Diagnosis is based on changes in the skin. Other tests may include:
There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms.
Scleroderma Research Foundation
Scleroderma Society of Canada
Arthritis Foundation website. Available at: http://www.arthritis.org .
Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. 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, Fisher MR, Champion HC, Housten-Harris T. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J . 2007;29:469-475.
Mayo Foundation for Medical Education and Research website. Available at: http://www.mayo.edu/ .
The Merck Manual of Medical Information . 17th ed. Simon and Schuster, Inc; 2000.
Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity . 2005;38:219-223.
Last reviewed February 2009 by
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.
Copyright © 2007 EBSCO Publishing All rights reserved.