SCLERODERMA

 Basic information

description

Scleroderma is a disease in which the skin and other body parts change gradually, becoming thick, stiff, and hardened. Scleroderma has two main classes, localized and systemic. Localized affects only certain parts of the body such as the skin. Systemic affects the whole body, including blood vessels and major organs. There are subgroups defined within these two classes. Scleroderma affects all age groups and females more than males. Localized form is more common in children and systemic form more often affects adults.

FREQUENT SIGNS & SYMPTOMS

• Patches on the skin that start in one place and spread.
• Fingers, toes, cheeks, nose, and ears may have numbness, pain, and color changes. Symptoms may be brought on by cold weather or emotional upset. This is called Raynaud phenomenon.
• Skin changes—thickened patches, especially on the fingers. Tight skin around the hands, face, or mouth.
• Digestive system problems—swallowing difficulty, poor food absorption, bloating after eating, weight loss, heartburn, and a feeling that food sticks in the chest.
• Feet and hands may be swollen.
• Feeling weak and tired.
• Joint pain, stiffness, and swelling.

causes

The exact cause is unknown. It is an autoimmune disorders. In these disorders, the immune system by mistake attacks the body itself. With scleroderma, it produces too much collagen, a fibrous type of protein found in connective tissues. Genetic and environmental factors may play a role also.

risk increases with

• Family history of scleroderma.
• Environmental risk factors are being researched.

PREVENTIVE MEASURES

Cannot be prevented at present.

expected outcomes

Outcome will depend on each individual patient and their symptoms. Many treatments are available.

possible complications

Complications can cause severe symptoms in the skin, gastrointestinal system, heart, lungs, and kidneys. Dental problems and sexual problems may develop.

 diagnosis & treatment

general measures

• Your health care provider will do a physical exam and check the affected skin. Questions will be asked about your symptoms. Medical tests may include blood and urine studies, heart and lung function tests, x-rays, CT, and others. A biopsy may be done (a small amount of tissue is removed and viewed under a microscope).
• There is no cure for the disorder. The goals of treatment are to help with the symptoms and prevent complications.
• Physical therapy and occupational therapy (to help with day-to-day living activities) may be prescribed.
• Wear warm clothing, such as heavy socks and gloves, if going out in the cold. Cover your head and face.
• Sleep on 2 or 3 pillows, or raise the head of your bed 5 to 8 inches to help prevent digestion problems.
• Stop smoking. Find a way to quit that works for you.
• Use heat to relieve joint stiffness.
• Seek counseling to help adjust to living with the disease. Joining a support group may help.
• To learn more: Scleroderma Foundation, 300 Rosewood Dr., Suite 105, Danvers, MA 01923; (800) 722-4673; website: www.scleroderma.org.

medications

• Different drugs may be prescribed, depending on your symptoms. Drugs can improve circulation, help joint stiffness and pain, aid digestive problems, improve the immune system, and lower high blood pressure.
• Use skin lotions, lubricants, and bath oils to soften skin. Other topical drugs may be prescribed.

activity

• Be as active as your strength permits; avoid fatigue.
• Regular exercise (or movement) helps keep skin flexible, helps blood circulation, and prevents fixed joints.

diet

Eat frequent, small meals to minimize bloating, heartburn, and stomach discomfort. A soft diet is sometimes helpful. Drink extra fluids to help with swallowing. A dietitian can help plan a nutritious diet.

 notify our office if

• You or a family member has scleroderma symptoms.
• Any sign of infection occurs.
• Symptoms get worse or new ones develop during treatment.

Special Notes: