scleroderma : Symptoms, Causes, Treatment & Prevention

What is Scleroderma?

Scleroderma (also known as systemic sclerosis) is a chronic disease that causes the skin to become thick and hard, a buildup of scar tissue, and damage to internal organs such as the heart and blood vessels, lungs, stomach and kidneys. The effects of scleroderma vary widely and range from minor to life-threatening, depending on how widespread the disease is and which parts of the body are affected.

There are two main types of scleroderma:

• localised scleroderma – just affects the skin
• systemic sclerosis – may affect blood circulation and internal organs as well as the skin

What causes Scleroderma?

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Doctors aren't certain what prompts this abnormal collagen production, but the body's immune system appears to play a role. In some genetically susceptible people, symptoms may be triggered by exposure to certain types of pesticides, epoxy resins or solvents.

What are the symptoms of Scleroderma?

Scleroderma's signs and symptoms vary, depending on which parts of your body are involved:

• Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.
• Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud's disease, this condition also occurs in people who don't have scleroderma.
• Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren't moving food properly through the intestines.
• Heart, lungs or kidneys. Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.

How is Scleroderma diagnosed?

The diagnostic process may require consultation with rheumatologists (arthritis specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests depending upon which organs are affected.

How is Scleroderma treated?

In some cases, the skin problems associated with scleroderma fade away on their own in three to five years. The type of scleroderma that affects internal organs usually worsens with time.

Medications:- No drug has been developed that can stop the underlying process of scleroderma — the overproduction of collagen. But a variety of medications can help control scleroderma symptoms or help prevent complications. To accomplish this, these drugs may:

• Dilate blood vessels. Blood pressure medications that dilate blood vessels may help prevent lung and kidney problems and may help treat Raynaud's disease.
• Suppress the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.
• Reduce stomach acid. Medications such as omeprazole (Prilosec) can relieve symptoms of acid reflux.
• Prevent infections. Antibiotic ointment, cleaning and protection from the cold may help prevent infection of fingertip ulcers caused by Raynaud's disease. Regular influenza and pneumonia vaccinations can help protect lungs that have been damaged by scleroderma.
• Relieve pain. If over-the-counter pain relievers don't help enough, you can ask your doctor to prescribe stronger medications.

Therapy:- Physical or occupational therapists can help you to:

• Manage pain
• Improve your strength and mobility
• Maintain independence with daily tasks

Surgery:- Used as a last resort, surgical options for scleroderma complications may include:

• Amputation. If finger ulcers caused by severe Raynaud's disease have developed gangrene, amputation may be necessary.
• Lung transplants. People who have developed high blood pressure in the arteries to their lungs (pulmonary hypertension) may be candidates for lung transplants.