Stem Cell Transplant Treats Rare Skin Disorder

NEW YORK (Reuters Health) - A man with a rare, potentially fatal skin disorder that was so severe that he could no longer eat is now symptom-free after receiving a transplant of his own stem cells, doctors in Texas report.

The disorder, scleromyxedema, is similar to a chronic connective tissue disease called scleroderma, which thickens the skin and causes it to become shiny and stiff. The cause of the condition is unknown, and treatment for the disease is often ineffective.

Six years before, the patient, a 46-year-old white man, had developed itchy spots on his hands that disappeared after treatment with oral and topical steroids. Three years later, the abnormalities returned as waxy, thickened skin. At that time the patient was diagnosed with scleromyxedema.

Despite treatment with steroids and other medications, the disease rapidly progressed during the next 2 years. Eventually, the man's face took on a ``cobblestone'' appearance, and he was not able to close his eyelids completely.

The side effects of two drugs used to treat the disorder--etanercept and interferon alfa-2b--were too much for the patient to handle, so doctors treated him with a therapy called photopheresis. This process involves exposing a patient's white blood cells to ultraviolet light and then returning them to the bloodstream. This restored the normal tightness of his skin, but the benefits were temporary.

By then, the disease had progressed to the point that the man was not able to eat and lost a significant amount of weight.

At that point, a team of doctors at the University of Texas M.D. Anderson Cancer Center in Houston led by Dr. Adrienne M. Feasel performed what is known as an autologous stem cell transplant on the patient.

After collecting stem cells--immature cells that can give rise to various specialized types of cells--from the man's bone marrow, the researchers wiped out his immune system with chemotherapy. They then transplanted the stem cells back into the man to reconstruct his immune system.

The approach seems to have worked, according to the report in the August issue of the journal Archives of Dermatology (news - web sites). Three months after the transplant, the cobblestone appearance of the man's face had disappeared and he was able to close his eyes and open his mouth. Since the procedure, he has gained more than 25 pounds.

``To our knowledge, this is the first report of treating scleromyxedema with transplantation, and additional investigations would be helpful for determining the response rate for progressive scleromyxedema,'' Feasel and her colleagues report.

SOURCE: Archives of Dermatology 2001;137:1071-1072.

Archived Stories by Date:

Aug 16 Aug 15 Aug 14 Aug 13 Aug 11 Aug 10 Aug 09 Aug 08 Aug 07 Aug 06 Aug 04 Aug 03 Aug 02 Aug 01 Jul 31 Jul 30 Jul 27 Jul 26 Jul 25 Jul 24 Jul 23 Jul 21 Jul 20 Jul 19 Jul 18

News Resources Message Boards: Post/Read Msgs Conversations: Start a live discussion News Alerts: human rights | Archives of Dermatology More Alerts: News Bulletins, News, Mobile, Stocks