Alternate Names: CREST syndrome; Progressive systemic sclerosis; Scleroderma

Causes and Risks: The cause of scleroderma is unknown. The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected. Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs is also thought to occur. In the skin, ulceration, calcification, and changes in pigmentation may occur. Systemic features may include fibrosis and atrophy of the heart, lungs, kidneys and gastrointestinal tract. 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. The incidence is 2 out of 10,000 people.

Prevention: There is no known prevention. Minimize 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

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

• The ESR is elevated.
• The rheumatoid factor is elevated.
• An antinuclear antibody test is positive.
• Urinalysis shows protein and microscopic blood.
• A chest X-ray may show fibrosis.
• Pulmonary function studies often show restrictive lung disease.
• A skin biopsy may also be performed.

• LE cell test
• febrile/cold agglutinins
• esophageal manometry
• ACE levels

Treatment: see scleroderma treatment

Support Groups: The stress of illness can often be helped by joining a support group where members share common experiences and problems. See systemic sclerosis (scleroderma) - support group.

Prognosis: In the majority of those affected, the disease is progressive. In some, remission occurs with a slow progression. People who only have skin involvement have a better prognosis (probable outcome). Death may occur from gastrointestinal, cardiac, kidney, or pulmonary (lung) involvement.

Complications:

• cardiac failure
• renal failure
• pulmonary fibrosis
• malabsorption (inadequate absorption of nutrients from the intestinal tract)

Call for an appointment with your health care provider if symptoms of this disorder are present.

Call your health care provider if you have scleroderma and symptoms become worse, or if new symptoms develop.