SYSTEMIC SCLEROS...

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Raynaud's phenomenon

Sclerodactyly

CREST syndrome

Telangiectasia

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, incidence, and risk factors

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

Signs 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

Reviewer Info: Ariel D. Teitel, MD, MBA, Chief, Division of Rheumatology, St. Vincent?s Hospital, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., 02/22/2009