Morphea (Question and Answer)

by Philip Clements, M.D., UCLA School of Medicine, Department of Rheumatology, (originally published in "Scleroderma Voice," Spring 2001)

Question: My 5-year-old daughter was diagnosed with morphea when she was 3. Due to the serious side effects, I've decided not to let her take any medication. Is there a possibility her disease will go away some day?

Also, the four fingers on her left hand are totally bent and tiny.

She has a waxy patch on the left side of her chest. Is there any test that can tell whether her deeper layers of skin are OK, and whether her chest will grow like normal women? Is it possible the morphea could turn into linear scleroderma?

Otherwise she seems healthy, eats well, and is smart.

Ms. L.W., San Gabriel, Calif.

Answer: Morphea is a type of scleroderma (hard skin) which affects the upper layer of skin but not the deeper layers of fat or muscle under the skin nor the internal or visceral organs. It typically appears as a thin, oval-shaped patch of thickening in the skin, often with a purple, brown, or white appearance and a red rim.

Linear scleroderma, another form of localized scleroderma, tends to affect not only the upper layer of skin but also the underlying fat and muscles. It presents as a "swath" of involvement down an extremity, one half of the face or one half of the torso.

Early on there may be darkening and swelling of the skin, but over time the involved tissue (skin, fat, muscle) may thin or atrophy.

If this involvement occurs over a joint or on the hand in a youngster, it may limit joint motion (produce a contracture or a deformity) and may retard growth of underlying bone.

Linear scleroderma tends to run a course which includes scarring and fibrosis, followed by atrophy. There is no clear consensus about whether there is any medication or treatment which can alter this course.

My supposition is that the young girl described has linear scleroderma affecting the left hand, arm, and chest, and she may also have a patch of morphea.

If you have not done so already, I suggest that you find a doctor who has experience in the scleroderma-spectrum diseases, so that an accurate determination can be made of the problem and its potential outcome and treatment. The Scleroderma Foundation can supply names of scleroderma-knowledgeable physicians in your area.