Scleroderma is a widespread autoimmune disorder. It causes the skin and other body parts to slowly degenerate, thicken, and stiffen. An autoimmune disorder is one in which the body produces antibodies against its own tissues for no known reason.
Scleroderma results from an overproduction of collagen, which is the main supportive protein in the body. The excess collagen affects tiny blood vessels and almost every organ.
A person may develop either a localized or a systemic form of scleroderma. The localized form is most common in children. It usually involves a few places on the skin, soft tissues, or muscles. The localized form rarely, if ever, progresses to the systemic form of scleroderma.
The systemic form affects skin, muscles, and soft tissues. It also affects other body organs and structures, such as the following: blood vesselsgastrointestinal (GI) tractheartkidneyslungs
Scleroderma can progress slowly, over many years. It often starts with the appearance of Raynaud's phenomenon. This condition causes abnormal sensitivity to cold in the fingers and toes. Swelling might be noted in the hands. Sweat secretion is decreased over involved areas of skin.
The skin becomes increasingly hard and dry. Extremities stiffen, and mobility is decreased. The mouth can become rigid and the face may look masklike and immobile. Body image can be negatively affected. Changes inside the body that are not visible may be just as important and threatening as those on the outside.
Some additional signs and symptoms of scleroderma include: calcium deposits in the skin and musclesdigestive problems, such as trouble swallowing, poor food absorption, bloating after eating, weight loss, and heartburn dilated blood vessels, especially on the hands, face, and chestheadaches high blood pressure joint pain or stiffness malaise, or a vague feeling of illnesspuffy hands and feet, particularly in the morningshortness of breathSjögren syndrome, an autoimmune disorder that causes dry eyes and mouthslow hardening and tightening of the skin, especially on the arms, face, and handssores or ulcers, especially on the fingers and toes, due to poor circulationswelling in the legs and anklesweakness
The exact cause of scleroderma is unknown. People heavily exposed to certain chemicals or substances may be at higher than normal risk for scleroderma. These substances include the following: detergentherbicidesplasticssilicasilicone prosthesesvarious drugs, such as cocaine and amphetamines
Mild cases may develop from occupational repetitive stresses on the hand, such as from working with a jackhammer. This condition usually occurs between the ages of 30 and 50, but it may also occur in children. It is four times more common in women than men but can affect either sex.
There is no known way to prevent scleroderma.
The diagnosis of scleroderma begins with a medical history and physical exam. Raynaud's phenomenon may be an early sign of scleroderma. A skin biopsy may help diagnose scleroderma. A tissue sample of the affected skin is removed for laboratory study. The healthcare professional may also order tests of heart, lung, and GI tract function.
There is no cure for scleroderma at this time. In a minority of people with the condition, problems involving the organs can cause: high blood pressure kidney failurelung problemsmalnutrition
Death may result from gastrointestinal, heart, kidney, or lung involvement.
Scleroderma is not contagious and poses no risk to others.
Treatment depends on the symptoms and severity of the scleroderma. Following is a list of some of the medications used to treat this disorder: angiotensin-converting enzyme (ACE) inhibitors to relax and dilate blood vesselsantacids and other medications to reduce heartburnantibiotics to fight infectionanti-rheumatic medications such as hydroxychloroquine (i.e., Plaquenil) or d-penicillamine. These reduce skin thickening and lung problems.calcium channel blockers to treat Raynaud's phenomenon corticosteroids, such as prednisone (i.e., Sterapred), to reduce inflammationimmunosuppressive medications, such as methotrexate (i.e., Rheumatrex, Trexall), azathioprine (i.e., Imuran, Azasan), cyclophosphamide (i.e., Cytoxan), and cyclosporine (i.e., Sandimmune, Neoral, Gengraf). These suppress the immune response to reduce inflammation.nitroglycerin ointment (i.e., Nitro-Bid) to treat hardened skinNSAIDs, such as ibuprofen (i.e., Advil, Motrin) and aspirin, to reduce inflammation
Psychotherapy or counseling may help the person adjust to living with an incurable disease. There are support groups for this disorder. Joining one may help the person to deal with the illness.
Medications used to treat scleroderma have many side effects. The side effects include the following: Antibiotics may cause stomach upset, diarrhea, and allergic reactions.Corticosteroids may cause weight gain, high blood pressure, bone loss or osteoporosis, an increased risk of infection, and muscle weakness.Antibiotics may cause stomach upset, diarrhea, and allergic reactions.Medications that suppress the immune system may cause an increased risk of infection, stomach upset, and liver or kidney damage.NSAIDs can cause stomach upset and a decrease in kidney function.
Calcium channel blockers have long been used to treat scleroderma as well as high blood pressure. However, the findings of two recent studies show that people who take a calcium channel blocker have a much higher incidence of complications than people taking other medications for high blood pressure. The findings of one study, for example, showed that the risk of heart attack was 27% greater. The risk of congestive heart failure was 26% higher in people taking calcium channel blockers. The American Heart Association recommends discussing the risks and benefits of the medication with a healthcare professional.
The course of scleroderma varies unpredictably. A person who only has the local form of scleroderma has a better prognosis. An individual with severe systemic scleroderma may have chronic lung, kidney, gastrointestinal, and cardiovascular problems.
A person with scleroderma should have frequent physical exams by his or her healthcare professional. This helps him or her monitor the activity of the disorder and determine possible complications. Frequent blood tests may help monitor the disorder as well. Any new or worsening symptoms should be reported to the healthcare professional.
The Merck Manual of Medical Information, Home edition, 1997
Professional Guide to Diseases, Sixth Edition. Springhouse: Springhouse Corporation, 1998.
Tierney, Lawrence, editor, "Current Medical Diagnosis and Treatment, 39th edition", 2000.
Griffith, H. Winter. Instructions for Patients. Philadelphia:W.B. Saunders company,1994
Understanding Autoimmune Disorders, [hyperLink url="http://www.niaid.nih.gov/publications/autoimmune.htm" linkTitle="www.niaid.nih.gov/publications/autoimmune.htm"]www.niaid.nih.gov/publications/autoimmune.htm[/hyperLink]