by Rick Alan
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 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.
Risk Factors TOP
Scleroderma is more common in women. The morphea type of scleroderma usually strikes people around 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 chance 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:
The doctor will ask 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:
There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms in various systems.
Joints and Muscles
Esophagus and Digestive Tract
Skin and Internal Organs TOP
Kidney Problems and/or High Blood Pressure TOP
Raynaud's Phenomenon TOP
There are no current guidelines to prevent scleroderma.
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 . Updated August 2012. Accessed August 21, 2013.
Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed.ebscohost.com/about/about-us . Updated August 5, 2013. Accessed August 21, 2013.
What is scleroderma? Scleroderma Foundation website. Available at: http://www.sclerod... . Accessed August 21, 2013.
Last reviewed May 2014 by Michael Woods, MD
Last Updated: 5/19/2014