Ed Dunscombe

My daughter is six-years old and has linear scleroderma. I first noticed it two years ago when a bluish area appeared to the right of her right eye. I first thought she had gotten into my wife's makeup! However I became concerned when of course it wouldn't wash off.

Our pedicatricians were stumped. A few months later we noticed a bald spot about the size of a nickel on top of her head. Later we saw a line descending down the top-right of her scalp, and also an affected area on her right-side temple. We first treated the bald spot as ringworm, and a local dermatologist treated with topical steroids for skin irritation. It wasn't until I asked him if the scalp and facial areas might not be related that he first mentioned scleroderma, or en coup de sabre.

I work in a library and started to research the condition. The standard medical sources had little. The Internet was better, and things like Medline and Health Index yielded much information too, as did the United Scleroderma Foundation.

We took her to see a second dermatologist and he recognized the condition. He put her on plaquenil for three months, and after that wanted to start her on prednisone. I wanted a second opinion before taking that route, and located a Dr. Thomas Lehman. He's head of the Dept. of Pediatric Rheumatology at the Hospital for Special Surgery in Manhattan. The hospital is usually rated in the top five in the US for rheumatology (US News & World Report annual survey of hospitals).

He saw her and recommended we start her immediately on a drug called methotrexate. He was careful to indicate the powerful nature of the drug and its potential side effects, but we felt we needed to rely on his expertise so went ahead with it. We now see him every 4-6 weeks at his other office at the Burke Rehab Center in White Plains, NY.

She has been on the drug now for thirteen months. The dosage has been cut in half since last Spring. She has bloodwork every week or two. I speak to Dr. Lehman's office weekly after they've checked the bloodwork, and they tell me whether she should take her dose (1 pill once a week) or skip it. Usually the bloodwork is fine. Once in a while he'll tell us to skip it for a week or two until the levels he check return to more normal rates.

The PDR on methotrexate is scary, but the PDR on most drugs is scary. So far she has exhibited no visible side effects whatsoever. The main concerns seem to be bone marrow suppression (and concurrent risk of infections), and kidney and liver compromise. Of course I worry continuously about the long-term effects of the drug, but feel the need to treat a condition I know she has outweighs the potential she may develop other problems later. Plus I'm impressed with Dr. Lehman and comforted by the fact that he specializes in kids.

When I reported back to the dermatologist that I was going with the methotrexate over his prednisone he was shocked and reported he'd never heard of treating with it for scleroderma. However I did find mention of it in the literature, and one of the scleroderma foundations stated that Dr. Lehman provides them with much of the information they give out on sclero. in children. I've also read that a Dr. Janet Pope in Canada is doing a study eveluating methotrextae for sclerodrma but that the reulsts won't be ready for a few more years.

My wife, Dr. Lehman and I agree that we see little change in her condition over the last year. He was hopeful that there might be a little regression. While I don't see any improvement I'm more than thankful that it at least seems to have stopped getting worse. Much of the affected area can be covered by her hair, and down the road a little makeup could also help. The area is the kind of thing you only notice after taking a second look.

So far the only problems she's had from other people have been from kids who of course are always quick to notice anything different. The only adult to mention it to her was one of the lab techs who draws her blood. One day she asked her what happened to her face, but more in a concerned than uncaring manner. I also have a lingering concern since the affected area is very close to her right eye, even extending onto the lid. However so far it has not affected her eye and Dr. Lehman seems to think it probably won't.

At the start of her treatment Dr. Lehman had her undergo a cat scan to make sure there was no internal involvement on her head, and he said the results were good. The cat scan was the worst of the treatment so far, requiring her to take a drug to knock her out so she'd be still for the test, then she was grumpy and dizzy for most of the day. The bloodwork was bad at first, but now she hardly notices. She and I always go out for breakfast at McDonald's after the bloodwork and before school (she has to fast 12 hours for the bloodwork). So we've made it into a little treat which perhaps makes up for some of it.

I'm constantly amazed at her ability to adapt to her problem. She knows she has a "condition." I think she feels it will one day get entirely better, but hope she develops the wisdom to be able to deal with it. My overriding questions have to do with the long-term effects of the drug, and whether or not the disease will "play itself out" after a few years so that we can wean her off the drug. Of course I have no way of knowing how much good it's done. Perhaps it was getting ready to stop on its own.

Dr. Lehman did leave her on the plaquenil until the Fall of 96. After her bloodwork was bad for a few weeks he decided to remove her from that and has left her off of it. That drug has the potential for causing eye damage. She saw an eye dr. every six months while she was on it, and there were no ill effects.

Dr. Lehman has a web page It mentions numerous rheumatological conditions but also goes into some detail on childhood scleroderma and related conditions.

I'd like to hear from other parents of kids with this disease, and/or anyone with experience taking methotrexate.

Ed Dunscombe: funscombe@carityconnect.com

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