What is scleroderma?
Scleroderma is a chronic disease that causes skin thickening and tightening, a build up of scar tissue, and damage to internal body organs.
What are the different types of systemic sclerosis?
Systemic sclerosis is classified into two main types:
(1) Limited cutaneous systemic sclerosis
- 70% of systemic sclerosis cases
- Affects only the face, forearms and lower legs up to the knee
- CREST syndrome (Calcinosis, Raynaud’s disease, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia) is an older term for limited scleroderma
(2) Diffuse cutaneous systemic sclerosis
- 30% of systemic sclerosis cases
- Involves the upper arms, thighs or trunk
What Causes Scleroderma?
• Studies of twins suggest it is not inherited.
• Scientists suspect that scleroderma comes from several factors:
- Abnormal immune or inflammatory activity
- Genetic makeup: Although genes seem to put certain people at risk for scleroderma and play a role in its course, the disease is not passed from parent to child like some genetic diseases.
- Environmental triggers: Research suggests that exposure to some environmental factors may trigger scleroderma-like disease in people who are genetically predisposed to it. Suspected triggers include viral infections, certain adhesive and coating materials, and organic solvents such as vinyl chloride or trichloroethylene.
- Hormones: By the middle-to-late childbearing years (age 30 to 55), women develop scleroderma 7 to 12 times more often than men. Because of female predominance at these and all ages, scientists suspect that hormonal differences between women and men play a part in the disease. However, the role of estrogen or other female hormones has not been proven.
What are the main symptoms and signs of scleroderma?
- Scleroderma is a variable, slow, and long-term disorder. It usually starts slowly, gradually gets worse over a few years, and then stabilizes.
- An early symptom of scleroderma is an extreme sensitivity to cold, called Raynaud’s phenomenon. In Raynaud’s phenomenon the blood vessels constrict or narrow. The narrowing of the blood vessels causes the blood supply to be greatly reduced; fingers and toes turn white and then blue. Often numbness and tingling are felt in the fingers and toes.
- Another early symptom of scleroderma is swelling of the hands and feet, especially in the morning. The skin of the fingers and toes may look and feel swollen. It might get shiny and the usual skin creases may disappear. The facial skin may seem stiff.
- Finger ulcers sometimes happen as a result of the narrowing of the small blood vessels. Some people will also develop small, white calcium deposits in various parts of the body.
- Scleroderma can also cause inflammation of the joints. This may be accompanied by pain, stiffness, swelling, warmth and tenderness.
- Muscle weakness is sometimes an early symptom of scleroderma.
- The disease may also affect the connective tissue of the internal organs. For example, if the digestive system is involved the muscles in the esophagus may become weak. Swallowing is affected so that it is difficult to get food down into the stomach. Heartburn can be a frequent problem.
- The lungs, heart and kidney are occasionally involved in scleroderma.
- Some develop severe lung disease, which comes in two forms: pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs).
- Common among people with scleroderma, heart problems include scarring and weakening of the heart (cardiomyopathy), inflamed heart muscle (myocarditis), and abnormal heartbeat (arrhythmia).
How Is Scleroderma Treated?
Currently, there is no treatment that controls or stops the underlying problem in all forms of scleroderma. Thus, treatment and management focus on relieving symptoms and limiting damage.
Here is a listing of the potential problems that can occur in systemic scleroderma and the medical and nonmedical treatments for them:
(1) Raynaud’s phenomenon:
- Dress warmly, with special attention to hands and feet.
- Dress in layers and try to stay indoors during cold weather.
- For severe cases, speak to your doctor about prescribing drugs called calcium channel blockers, which can open up small blood vessels and improve circulation.
(2) Skin sores or ulcers:
- Increasing your dose of calcium channel blockers
- Applying nitroglycerine paste or antibiotic cream.
- Severe ulcerations on the fingertips can be treated with skin graft.
(3) Stiff and painful joints:
- Stretching exercises under the direction of a physical or occupational therapist are extremely important to prevent loss of joint motion.
- Exercise regularly.
- Use acetaminophen or nonsteroidal anti-inflammatory drug.
(4) Skin disease:
- Apply oil-based creams and lotions frequently, and always right after bathing.
- Apply sunscreen before you venture outdoors to protect against further damage from the sun’s rays.
- 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.
(5) Dry mouth and dental problems:
- Brush and floss your teeth regularly.
- Have regular dental checkups.
- Keep your mouth moist by drinking plenty of water, sucking ice chips, using sugarless gum and hard candy.
- If dry mouth still bothers you, ask your doctor about a saliva substitute or prescription medications such as pilocarpine hydrochloride that can stimulate the flow of saliva.
(6) Gastrointestinal problems:
Eat small, frequent meals.
- To keep stomach contents from backing up into the esophagus, stand or sit for at least an hour after eating.
- Avoid late-night meals, spicy or fatty foods, and caffeine.
- Oral antibiotics may stop bacterial overgrowth in the bowel, which can be a cause of diarrhea in some people with systemic sclerosis.
(7) Lung damage:
- Pulmonary fibrosis may be treated with drugs that suppress the immune system, such as cyclophosphamide or azathioprine, along with low doses of corticosteroids.
- Pulmonary hypertension may be treated with drugs that dilate the blood vessels, such as prostacyclin.
(8) Heart problems:
- Treatment ranges from drugs to surgery and varies depending on the nature of the condition.
(9) Kidney problems:
- Renal crisis occurs in about 10%t of all patients with scleroderma, primarily those with early diffuse scleroderma. Renal crisis results in severe uncontrolled high blood pressure, which can quickly lead to kidney failure. Check your blood pressure regularly.
- If you have kidney problems, take your prescribed medications faithfully.
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