SCLERODERMA

General Information

DEFINITION--A rare, connective tissue disease in which the skin and other body parts gradually degenerate, thicken and become stiff (sclero means thickening and derma means skin).

BODY PARTS INVOLVED--Skin; joints; digestive system, especially the esophagus; intestinal tract; heart; kidneys; lungs; blood vessels; fingers; toes.

SEX OR AGE MOST AFFECTED--Adults of both sexes, but more common in women between ages 30 and 50.

> Fingers--

hardening and thickening of the skin, stiffness, poor circulation, numbness and fingertip ulceration.

• Digestive system--swallowing difficulty, poor food absorption, bloating after eating, weight loss, heartburn and a feeling that food sticks in the chest.
• Skin--hardening and thickening, especially in the face, which becomes tight and loses its elasticity.
• Muscle aches; weakness and fatigue.
• Joint pain, stiffness and swelling; anemia.

CAUSES--Unknown, but may be an autoimmune disorder in which the body's immune system attacks its own tissues. The connective tissue (the framework for all body tissues and blood vessels) thickens, becoming stiff and inflexible.

RISK INCREASES WITH--Unknown.

HOW TO PREVENT--Cannot be prevented.

What To Expect

DIAGNOSTIC MEASURES--

• Medical history and exam by a doctor.
• Laboratory blood tests to detect anemia and measure antibodies.
• Urinalysis to detect red cells in the urine.
• ECG (See Glossary), lung function tests; barium enema; and skin biopsy (See Glossary).
• X-rays of the hands, esophagus and chest.

APPROPRIATE HEALTH CARE

• Self-care during treatment.
• Treatment program will vary.
• Psychotherapy or counseling to adjust to living with an incurable disease.
• Home care usually. Rarely, hospitalization for heart, lung or kidney complications or for surgical procedure (e.g., on the esophagus).

POSSIBLE COMPLICATIONS

• Bleeding tendencies.
• Heart-rhythm disturbances.
• Congestive heart failure.
• Kidney failure.
• High blood pressure.
• Lung destruction.
• Poor wound healing and gangrene.

PROBABLE OUTCOME--The course of the disorder is variable and unpredictable. It is often slowly progressive and affects the heart, lungs and kidneys. In some, the disease may remain limited and nonprogressive for long periods of time.

How To Treat

GENERAL MEASURES--

• Because of poor circulation, wear warm clothing, especially socks and gloves. Avoid exposure to extreme cold.
• Protect yourself from burns and cuts.
• Sleep on 2 or 3 pillows, or raise the head of your bed 5 to 8 inches to prevent stomach acid from rolling back into the esophagus.
• Learn biofeedback techniques to increase circulation to the extremities.
• Don't smoke.
• Use heat to relieve joint stiffness.
• See Resources for Additional Information.

MEDICATION--

• You may take non-prescription antacids to relieve heartburn or indigestion, and aspirin or ibuprofen for muscle aches and joint pain.
• Use lotions, lubricants and bath oil on skin.
• Your doctor may prescribe cortisone drugs to relieve inflammatory symptoms and antibiotics to fight infections. There is no one drug that is helpful for this disorder.

ACTIVITY--

• Be as active as your strength permits; avoid fatigue.
• Regular exercise (or movement) can help keep the skin flexible, maintain good blood circulation and prevent fixed joints. Physical therapy may help preserve muscle strength. Ask your doctor.

DIET--Eat frequent, small meals to minimize bloating, heartburn and gastrointestinal discomfort. A soft diet is sometimes recommended. Use additional fluids to help with swallowing. Consult a dietitian.

Call Your Doctor If

The following occurs during treatment: Unexplained bruising or bleeding under skin. Slow healing of a wound.