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Raynaud's pheno...

CREST syndrome

Sclerodactyly

Telangiectasia

 

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; Localized scleroderma

Causes

The cause of scleroderma is unknown. People 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 get scleroderma more often than men do. Risk factors include occupational exposure to silica dust and polyvinyl chloride.

Symptoms

Skin symptoms may include:

• Blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
• Hair loss
• Skin hardness
• Skin is abnormally dark or light
• Skin thickening and shiny hands and forearm
• Small white lumps beneath the skin
• Tight and mask-like facial skin
• Ulcerations on fingertips or toes

Bone and muscle symptoms may include:

• Joint pain
• Numbness and pain in the feet
• Pain, stiffness, and swelling of fingers and joints
• Wrist pain

Breathing problems may include:

• Dry Cough
• Shortness of breath
• Wheezing

Digestive tract problems may include:

• Bloating after meals
• Constipation
• Diarrhea
• Difficulty swallowing
• Esophageal reflux or heartburn
• Weight loss

Additional symptoms associated with this disease include:

• Eye burning, itching, and discharge

Exams and Tests

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

Tests may include:

• Antinuclear antibody
• Chest x-ray
• CT scan of the lungs
• Echocardiogram
• ESR
• Rheumatoid factor
• Urinalysis
• Pulmonary function studies
• Skin biopsy

Treatment

Drugs used to treat scleroderma include:

• Corticosteroids
• Immunosuppressants (Methotrexate, Cytoxan)
• Nonsteroidal anti-inflammatory drugs (NSAIDs)

Other treatments for specific symptoms may include:

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

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 - resources

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, heart, kidney, or lung involvement.

One type of scleroderma, called localized scleroderma, involves only problems of the skin of the hands and face. It gets worse very slowly and usually does not affect any other parts of the body.

Systemic scleroderma can involve many organs in the body. In some people, it will progress slowly and not involve any organs in the body. In others, organs such as the lungs, kidneys, intestines, gallbladder, and heart become involved.

For some, symptoms and problems develop quickly over the first few years, and continue to worsen. Others get worse much more slowly. Problems with the lungs are the most common cause of death in patients with scleroderma.

Possible Complications

• Heart failure
• Kidney failure
• Malabsorption
• Pulmonary fibrosis (the most common cause of death in people with scleroderma)
• Pulmonary hypertension

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.

References

Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.

Harris ED Jr., Budd RC, Genovese MC, Firestein GS, Sargent JS, eds. Kelley's Textbook of Rheumatology. 7th ed. Philadelphia, Pa: Saunders Elsevier; 2005.

Clouse RE, Diamant NE. Esophageal Motor and Sensory Function and Motor Disorders of the Esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006: chap 41.

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