Scleroderma

Definition

Scleroderma is a disease that involves the buildup of scar-like tissue in the skin. It also damages the cells that line the walls of the small arteries.

Alternative Names

CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Mixed connective disease; Morphea - linear

Causes

Scleroderma is a type of autoimmune disorder in which the immune system mistakenly attacks and destroys healthy body tissue.

The cause of scleroderma is unknown. A buildup of a substance called collagen in the skin and other organs leads to the symptoms of the disease.

The disease most often affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.

Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis.These cases are referred to as mixed connective disease.

Symptoms

Some types of scleroderma affect only the skin, while others affect the whole body.

Skin symptoms of scleroderma may include:

Bone and muscle symptoms may include:

Breathing problems may result from scarring in the lungs and can include:

Digestive tract problems may include:

Exams and Tests

The health care provider will do a physical exam. The exam may show tight, thick skin.

Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become inflamed. Problems with your kidneys can lead to high blood pressure.

Blood and urine tests may include:

Other tests may include:

Treatment

There is no specific treatment for scleroderma.

Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.

Medicines used to treat scleroderma include:

Other treatments for specific symptoms may include:

Treatment often involves physical therapy as well.

Support Groups

Some people can benefit from attending a support group for people with scleroderma.

Outlook (Prognosis)

In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease slowly gets worse.

People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.

When to Contact a Medical Professional

Call your health care provider if your symptoms become worse or new symptoms develop.

References

Varga J. Systemic Sclerosis (Scleroderma). In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 275.

Varga J. Etiology and Pathogenesis of Scleroderma.