Health Encyclopedia

Scleroderma

Definition

Scleroderma is a widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs.

Alternative Names

CREST syndrome; Progressive systemic sclerosis; Systemic sclerosis

Causes, incidence, and risk factors

The cause of scleroderma is unknown. Persons with this condition have a build up of a substance called collagen in the skin and other organs. This build up leads to the symptoms associated with the disease.

The disease usually affects people 30 to 50 years old. Women are affected more often than men. Risk factors are occupational exposure to silica dust and polyvinyl chloride.

Symptoms

Blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
Pain, stiffness, and swelling of fingers and joints
Skin thickening and shiny hands and forearm
Skin is hard
Tight and mask-like facial skin
Ulcerations on fingertips or toes
Esophageal reflux or heartburn
Difficulty swallowing
Bloating after meals
Weight loss
Diarrhea
Constipation
Shortness of breath

Additional symptoms that may be associated with this disease:

Wrist pain
Wheezing
Skin, abnormally dark or light
Joint pain
Hair loss
Eye burning, itching, and discharge

Signs and tests

Examination of the skin may show tightness, thickening, and hardening.

Tests may include:

Treatment

Drugs used to treat scleroderma include:

Nonsteroidal anti-inflammatory drugs (NSAIDs)
Corticosteroids

Other treatments for specific symptoms may include:

Antacids for heartburn
Medications to treat Raynaud's phenomenon
Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems
Medicines to improve breathing

Treatment usually includes a combination of physical therapy and skin and joint protection techniques (for example, avoiding cold in the case of Raynaud's phenomenon).

Support Groups

See: Scleroderma - support group

Expectations (prognosis)

In most patients, the disease slowly gets worse. People who only have skin involvement have a better outlook. Death may occur from gastrointestinal, cardiac, kidney, or pulmonary (lung) involvement.

Complications

Calling your health care provider

Call for an appointment with your health care provider if:

You have symptoms of scleroderma
You have scleroderma and symptoms become worse or new symptoms develop

Prevention

There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.

Review Date:4/26/2007
Reviewed By:Steve Lee, DO, Rheumatology Fellow, Loma Linda University Medical Center, Loma Linda, CA. Review provided by VeriMed Healthcare Network.

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