CREST syndrome

Definition

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

Alternative Names

Causes

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

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

Exams and Tests

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

Tests may include:

• ESR
• Rheumatoid factor
• Antinuclear antibody
• Urinalysis
• Chest x-ray
• Pulmonary function studies
• Skin biopsy

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

Outlook (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.

Possible Complications

• Heart failure
• Kidney failure
• Pulmonary fibrosis
• Pulmonary hypertension
• Malabsorption

When to Contact a Medical Professional

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.

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