FAMILY MEDICINE® COLUMN

By Martha A. Simpson, D.O., M.B.A.
Associate Professor of Family Medicine
Ohio University College of Osteopathic Medicine

DAUGHTER’S MORPHIA SCLERODERMA IS RARE CONNECTIVE TISSUE DISEASE

Question: My 15-year-old daughter was diagnosed with morphia scleroderma about three years ago.  She has a large discoloration that looks like a scar that the doctor says is associated with the disease. It’s on the side of her stomach -- about five inches long and about an inch and a half wide.  The doctor has prescribed creams to help it fade but nothing seems to work.  It’s embarrassing for her when she wears bathing suits in the summertime. She wants to go to a skin doctor to see about having it removed.  Can you tell me if this is possible? 

Answer: There are several different types of scleroderma. Before I discuss the specifics of the morphia type, let me tell you a little bit about the disease in general. Scleroderma can cause thickening, hardening or tightening of the skin, blood vessels and internal organs. It is classified as an autoimmune disorder, because the sufferer’s connective tissue is attacked by his or her own immune system. This occurs when the immune system mistakes the body’s connective tissue for a foreign invader. Connective tissue is found throughout the body, providing support and form for organs and structures.

When the immune system attacks the connective tissue, researchers believe, it causes an overproduction of collagen. This is a tough, hard protein that your body uses to make up all connective tissue, including tendons, bones, ligaments and skin. It is when this excess collagen is deposited in various places throughout the body that the hardening and stiffening of tissues that I mentioned earlier takes place.

Morphia scleroderma is a rare form of what’s called localized scleroderma, which means it doesn’t affect internal organs. It occurs in children as well as adults and produces hard, scar-like oval shaped patches on the skin, usually on the trunk and limbs. The plaques start off purple in color then become ivory white in the middle with a light purple border. Old lesions may be brown. The surface is smooth, shiny and hairless. The discoloration of the skin is not just “associated with the disease” -- as you put it -- but it is the condition itself. Therefore, surgical removal is not really an option.

As for treatment, there are many treatments that have been tried, but none have been proven to be effective in most cases. Also, there are more treatment options available for adults than for children. There has been some success with the use of the anti-cancer drug methotrexate in children with localized skin lesions, but more studies are needed.

Here are some other treatments that have had some success, but may not be indicated for children. There are numerous creams that have been tried with varying success, including steroid creams. A variety of phototherapy techniques have been tried with different types of light. One that has been successful for some people is long wave ultraviolet A, with and without the use of photosensitive creams. In other cases, long-term courses of antibiotics, oral steroids, and the seizure medication Dilantin have been tried and have been found to be helpful.

Because this is a very rare condition, probably the most important thought I can leave you with is to find a physician who specializes in the treatment of juvenile morphia scleroderma. He or she will be able to give you the best advice for the treatment, both short- and long-term, for your daughter.

Family Medicine® is a weekly column. To submit questions, write to Martha A. Simpson, D.O., M.B.A., Ohio University College of Osteopathic Medicine, P.O. Box 110, Athens, Ohio 45701, or via e-mail to readerquestions@familymedicinenews.org. Medical information in this column is provided as an educational service only. It does not replace the judgment of your personal physician, who should be relied on to diagnose and recommend treatment for any medical conditions. Past columns are available online at www.familymedicinenews.org.