Scleroderma

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Scleroderma is an autoimmune disease of the connective tissue. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system. It is characterized by the formation of scar tissue in the skin and organs of the body. This leads to thickness and firmness of involved areas. When it's diffuse or widespread over the body, is also referred to as systemic sclerosis.

Symptoms:

Gradual hardening, thickening & tightening of the skin

Skin discoloration

Numbness of extremities

Shiny skin

Small white lumps under the surface of the skin

Cause:
The cause is not known. Researchers have found some evidence that genes are important factors, but the environment seems to also play a role. The result is activation of the immune system, causing injury to tissues that result in injury similar to scar tissue formation. The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not unusual to find other autoimmune diseases in families of scleroderma patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.

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Treatment:
Currently, there is no treatment that controls or stops the underlying problem--the overproduction of collagen--in all forms of scleroderma. Thus, treatment and management focus on relieving symptoms and limiting damage. Your treatment will depend on the particular problems you are having. Some treatments will be prescribed or given by your physician. Others are things you can do on your own.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors involved in your care. Typically, care will be managed by a rheumatologist, who may refer you to other specialists, depending on the specific problems you are having: for example, a dermatologist for the treatment of skin symptoms, a nephrologist for kidney complications, a cardiologist for heart complications, a gastroenterologist for problems of the digestive tract, and a pulmonary specialist for lung involvement.

Here are some of the potential problems that can occur in systemic scleroderma and the medical and nonmedical treatments for them.

. . . Raynaud's Phenomenon:
One of the most common problems associated with scleroderma, Raynaud's phenomenon can be uncomfortable and can lead to painful skin ulcers on the fingertips. Smoking makes the condition worse. The following measures may make you more comfortable and help prevent problems:

Don't smoke! Smoking narrows the blood vessels even more and makes Raynaud's phenomenon worse.

Dress warmly, with special attention to hands and feet. Dress in layers and try to stay indoors during cold weather.

Use biofeedback and relaxation exercises

For severe cases, speak to your doctor about prescribing drugs called calcium channel blockers, such as nifedipine (Procardia), which can open up small blood vessels and improve circulation.

If Raynaud's leads to skin sores or ulcers, increasing your dose of calcium channel blockers may help. You can also protect skin ulcers from further injury or infection by applying nitroglycerine paste or antibiotic cream.

. . . Stiff, Painful Joints:
Hand joints can stiffen because of hardened skin around the joints or inflammation of the joints themselves. Other joints can also become stiff and swollen. The following may help:

Exercise regularly. Swimming can help maintain muscle strength, flexibility, and joint mobility.

Use acetaminophen or an over-the-counter or prescription nonsteroidal anti-inflammatory drug, as recommended by your doctor, to help relieve joint or muscle pain.

Learn to do things in a new way that will put less stress on tender joints.

. . . Skin Problems:
When too much collagen builds up in the skin, it crowds out sweat and oil glands, causing the skin to become dry and stiff. To ease dry skin, try the following:

Apply oil-based creams and lotions frequently, and always right after bathing.

Apply sunscreen before you venture outdoors.

Use humidifiers to moisten the air in your home in colder winter climates.

Avoid very hot baths and showers, as hot water dries the skin.

Avoid harsh soaps, household cleaners, and caustic chemicals.

Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.

. . . Dry Mouth and Dental Problems:
Dental problems are common in people with scleroderma for a number of reasons: tightening facial skin can make the mouth opening smaller and narrower, which makes it hard to care for teeth; dry mouth due to salivary gland damage speeds up tooth decay; and damage to connective tissues in the mouth can lead to loose teeth. You can avoid tooth and gum problems in several ways:

Brush and floss your teeth regularly.

Have regular dental checkups.

If decay is a problem, ask your dentist about fluoride rinses or prescription toothpastes that remineralize and harden tooth enamel.

Consult a physical therapist about facial exercises to help keep your mouth and face more flexible.

Keep your mouth moist by drinking plenty of water, sucking ice chips, using sugarless gum and hard candy, and avoiding mouthwashes with alcohol.

. . . Gastrointestinal Problems:
Systemic sclerosis can affect any part of the digestive system. As a result, you may experience problems such as heartburn, difficulty swallowing, the feeling of being full after you've barely started eating, or intestinal complaints such as diarrhea, constipation, and gas. In cases where the intestines are damaged, your body may have difficulty absorbing nutrients from food. Although GI problems are diverse, here are some things that might help at least some of the problems you have:

Eat small, frequent meals.

Raise the head of your bed with blocks, and stand or sit for one to three hours after eating to keep stomach contents from backing up into the esophagus.

Avoid late-night meals, spicy or fatty foods, and alcohol and caffeine.

Chew foods well and eat moist, soft foods.

Ask your doctor about prescription medications for problems such as diarrhea, constipation, and heartburn.

. . . Lung Damage:
About 10 to 15 percent of people with systemic sclerosis develop severe lung disease, which comes in two forms: pulmonary fibrosis and pulmonary hypertension. Treatment for the two conditions is different.

Pulmonary fibrosis may be treated with drugs that suppress the immune system such as cyclophosphamide (Cytoxan or Imuran), along with low doses of corticosteroids.

Pulmonary hypertension may be treated with drugs that dilate the blood vessels such as prostacyclin (Iloprost).

. . . Heart Problems:
About 15 to 20 percent of people with systemic sclerosis develop heart problems, including scarring and weakening of the heart, inflamed heart muscle, and abnormal heart beat. All of these problems can be treated. Treatment ranges from drugs to surgery, and varies depending on the nature of the condition.

. . . Kidney Problems:
About 15 to 20 percent of people develop severe kidney problems, including loss of kidney function. Because uncontrolled high blood pressure can quickly lead to kidney failure, it's important that you take measures to minimize the problem. Things you can do:

Check your blood pressure regularly and, if you find it to be high, call your doctor right away.

Take your prescribed medications faithfully. In the past two decades, drugs known as ACE inhibitors, including Capoten, Vasotec, and Accupril, have made scleroderma-related kidney failure a less-threatening problem than it was in the past.

. . . Cosmetic Problems:
Even if scleroderma doesn't cause any lasting physical disability, its effects on the skin's appearance--particularly on the face--can take their toll on your self-esteem. Fortunately, there are procedures to correct some of the cosmetic problems scleroderma causes.

The appearance of small red spots on the hands and face caused by swelling of tiny blood vessels beneath the skin, may be lessened or even eliminated with the use of lasers.

Facial changes that may run down the forehead, may be corrected through cosmetic surgery.

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