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Scleroderma by Rick Alan

En Español (Spanish Version) More InDepth Information on This Condition

Definition

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:

Localized scleroderma (also known as morphea)—usually affects only the skin in isolated parts of the body. This form is less serious.
Systemic scleroderma—affects widespread areas of skin and/or internal organs, most often the lungs (Certain categories of this form of scleroderma are more serious and can be fatal.)
Overlap syndrome—may involve features of scleroderma and features of other autoimmune syndromes

Causes

Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma. 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.

Risk Factors

These factors increase your chance of developing scleroderma. Tell your doctor if you have any of these risk factors:

Sex: Female
Age: 30-50 years
Occupational exposures (such as, polyvinyl chloride or silica dust)
Genetic predisposition

Symptoms

Localized Scleroderma

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:

Hard patches on the skin, most often on the face or trunk (morphea)
Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)

Systemic Scleroderma

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. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.

Raynaud's Phenomenon Symptom

Low blood flwo to fingers, vasoconstriction

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:

Diffuse thickening and hardening of the skin
Joint and muscular pain, stiffness, and swelling
Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
Inflammation and thickening of large and small blood vessels

Complications of diffuse scleroderma can affect virtually every system of the body. Prominent complications include:

Diagnosis

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:

Blood tests
Esophagus motility study
X-ray—a test that uses radiation to take a picture of structures inside the body, especially bones
MRI scan—a test that uses magnetic waves to make pictures of structures inside the body
CT scan—a type of x-ray that uses a computer to make pictures of structures inside the body
Biopsies of skin and other tissues—removal of a sample of tissue to test

Treatment

There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms.

Joints and Muscles

Aspirin
Nonsteroidal anti-inflammatory drugs
Corticosteroids, or prednisone (Deltasone, Orasone, Prednicen-M)

Esophagus and Digestive Tract

Medicines to limit acid production in the stomach
Small, frequent meals
Sleeping with your head elevated to avoid acid reflux

Skin and Internal Organs

Corticosteroids
Immune suppressants, or azathioprine (Imuran, Azasan)
Phototherapy

Kidney Problems and/or High Blood Pressure

Angiotensin-converting enzyme (ACE) inhibitors and other antihypertensive drugs
Gleevec (imatinib mesylate)—already approved for the treatment of certain leukemias; used in several trials and may inhibit the production of fibrosis in scleroderma
Pulmonary hypertension treatment (such as, bosentan, sildenafil)

Raynaud's Phenomenon

Calcium channel blockers, like nifedipine (Adalat, Procardia, Afeditab, Nifediac)—to dilate blood vessels in the extremities
Proper shelter and clothing to avoid cold
Not smoking

Restricted Mobility

Physical therapy and exercise to maintain circulation, joint flexibility, and muscle strength

Prevention

There are no guidelines for preventing scleroderma because the cause is unknown.

RESOURCES:

Scleroderma Foundation http://www.scleroderma.org
Scleroderma Research Foundation http://www.srfcure.org

CANADIAN RESOURCES:

Arthritis Society http://www.arthritis.ca
Scleroderma Society of Canada http://www.scleroderma.ca

References:

Arthritis Foundation website. Available at: http://www.arthritis.org. Accessed July 9, 2009.
Firestein ED et al. Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia: Saunders, 2008.
Habif TP. Clinical Dermatology. 4th ed. St. Louis: Mosby, 2004.
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.
Mayo Foundation for Medical Education and Research website. Available at: http://www.mayo.edu. Accessed July 9, 2009.
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 September 2011 by Rosalyn Carson-DeWitt, MD

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