Pain Management: Scleroderma

Scleroderma is a chronic autoimmune disorder that can cause tightening and thickening of the skin, thickening of blood vessels, and damage to internal organs. The disease usually affects adults between the ages of 30 and 50 and affects more women than men.

There are two types of scleroderma: Localized scleroderma and generalized scleroderma. Localized scleroderma affects mainly the skin, while generalized scleroderma is a systemic disease that may affect many parts of the body.

There are two kinds of localized scleroderma:

• Morphea: a condition involving hard, oval-shaped patches on the skin. Patches start out red or purple and then turn whitish in the center. Rarely, this type can affect blood vessels or internal organs (generalized morphea).
• Linear: a condition involving lines or streaks of thickened skin on the arms, legs or face.

There are two kinds of generalized scleroderma:

• Limited scleroderma: Occurs gradually, and affects the skin of the face, hands and feet with possible damage to the esophagus, lungs, or intestines as well.
• Diffuse scleroderma: Occurs with a more rapid onset and involves thickening of the skin of the trunk (middle part of body), thighs and upper arms in addition to the hands and feet. Diffuse scleroderma also affects internal organs (such as the heart, lungs or kidneys) more often.

What Causes Scleroderma?

The cause of scleroderma is not known, but it is one of a group of systemic autoimmune diseases in which the body's own immune system appears to contribute to inflammation of skin and other organs. Doctors know that scleroderma causes the body to make too much collagen (a protein) that can result in thickening or tightening of skin and scarring of internal organs. In addition, abnormal spasm and thickening of blood vessels contributes to tissue damage and may cause abnormally elevated pressure in systemic and pulmonary arteries.

What Are the Symptoms of Scleroderma?

Symptoms of scleroderma may affect the skin, joints and other organs and include:

• Hardening/thickening of the skin, especially skin of the hands and/or face, causing a shiny and creaseless appearance
• Cold sensitivity of fingers with red, white, and blue discoloration (called Raynaud's phenomenon)
• Ulcers/sores on finger tips
• Small red spots on the face and chest (called telangiectasias)
• Puffiness/swelling or pain in fingers and/or toes
• Pain or swelling of joints
• Muscle weakness
• Dryness of eyes/mouth (called Sjögren's syndrome)
• Edema (swelling)
• Shortness of breath
• Heartburn
• Diarrhea
• Weight Loss

How Is Scleroderma Diagnosed?

This condition is diagnosed by physical exam, X-ray studies, and blood tests. Evaluation may include tests of the heart, lungs, and esophagus or a possible skin biopsy.

How Is Scleroderma Treated?

Many different treatments are available to help control scleroderma. Medications used to treat the condition include:

• NSAIDs (nonsteroidal anti-inflammatory drugs like ibuprofen or aspirin) for inflammation and pain
• Steroids and other immune suppressants to decrease inflammation and treat muscle and joint problems or involvement of internal organs
• Medications to improve blood flow to the fingers
• blood pressure control medication
• Medications to treat elevated pressure in the arteries of the lungs
• Heartburn medication

Other treatments include:

• Exercise to improve overall health
• Skin and joint protection
• Stress management to help people deal with this chronic disease
• If severe organ damage occurs, organ transplantation may be necessary.