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SCLERODERMA USUALLY AFFECTS THE SKIN FIRST

Q: My daughter, age 38 and healthy all her life, has just been diagnosed with scleroderma. I know that it attacks the joint tissues and hardens them. Please tell me more details, what the long-term prognosis is and what treatments there are.

Her first symptom was a white, scar-like spot on her back the size of a half-dollar, discovered in late summer. She has been losing her hair and has had heartburn and diarrhea. She was first treated for irritable bowel syndrome. Then they did a biopsy of this place on her back and discovered the scleroderma.

-- M.W., Springfield, Mo.
A: Scleroderma is a relatively rare but quite serious disease, and I know this is a difficult time for your daughter.

Scleroderma is a chronic connective tissue disorder characterized by degenerative changes and scarring on the skin and in the joints and internal organs. The cause is not known, but it's probably a disease of the immune system. Workplace exposure to silica dust has also been implicated.

Most of the time, changes are seen in the skin first, such as progressive, plaque-like thickening and discoloration. Other early symptoms include itching and difficulty opening the mouth and swallowing.

Symptoms usually begin to appear between the ages of 30 and 50, and women are two to three times more often affected than men are.

Scleroderma is really two separate but related conditions, either localized or systemic. Localized scleroderma is usually confined to the skin and is not life-threatening. Systemic scleroderma is more serious and is characterized as either being limited or diffuse. People with limited systemic scleroderma usually have a combination of symptoms, mostly but not entirely related to the skin.

Diffuse systemic scleroderma results in the rapid progression of the disease to internal organs. This can eventually affect the gastrointestinal tract, lungs, heart and kidneys. At this point, scleroderma is a life-threatening disease.

The course of the disease is variable and unpredictable, but the amount of involvement within the first few years of the disease indicates how bad it will get. Although some damage to internal organs is almost inevitable, sometimes years go by before this damage is seen.

Although prognosis is improving, there is no known cure. Treatment is limited to relief of symptoms and general care of the organ system under the most severe attack.

For example, non-steroidal anti-inflammatory drugs (NSAIDs) and steroids can relieve joint pain and weakness. Physical therapy and exercise can help maintain muscle strength.

Penicillamine has been shown to be helpful for those at high risk of internal organ involvement. Immunosuppressive drugs can help some people.

Eating small meals and taking antacids and histamine-blocking drugs to inhibit stomach acid production can help with the heartburn. Sleeping with the head elevated can also help.

Because of the national concern about HIV and AIDS, researchers working on diseases of the immune system have gotten additional resources that may result in meaningful scientific breakthroughs. Let us hope this happens soon for scleroderma and brings relief to your daughter and others with this terrible disease.

Update on diarrhea: One of the most common causes of diarrhea is the effect of antibiotics on the intestines. This is true for everyone, but is more of a problem with children -- 40 percent of children taking antibiotics will develop diarrhea.

A study recently published in the Journal of Pediatrics shows that a simple, very safe and inexpensive treatment can dramatically reduce diarrhea caused by using antibiotics.

Among people taking a capsule containing the beneficial bacterium Lactobacillis each day of antibiotic therapy, the rate of diarrhea was cut by two-thirds and the length of time for those who did have some was shortened as well.

Lactobacillis is naturally found in dairy products, but processing kills most if not all of it. However, many grocery stores do carry milk or milk products that have been fortified with Lactobacillis.

Dr. Allen Douma welcomes questions from readers. Although he cannot respond to each one individually, he will answer those of general interest in his column. Write to Douma in care of Tribune Media Services, 435 N. Michigan Ave., Suite 1400, Chicago, Ill. 60611. His e-mail address is DRFamily@aol.com.

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