A definition of Juvenile Scleroderma in simple, easy to understand language

My Child Has Linear Scleroderma en coupe de sabre – What Does This Mean?
Written by Thomas J.A. Lehman, M.D.
Chief Pediatric Rheumatologist
New York, NY

Linear scleroderma may take many different forms. Linear scleroderma en coupe de sabre is one of the most disturbing because it occurs on the face and is easily seen. However, except for its easy visibility, it is often one of the mildest forms of linear scleroderma. It is called en coupe de sabre because its appearance is similar to the scar many foot soldiers had when they had been struck on the head with a sword (sabre in French) by an opposing cavalry soldier.

For many children the area of thickened skin extends from the hairline onto the forehead. It may be just to one side of the midline, or off to one side by the eye. The majority of children with linear scleroderma have no other findings. Involvement of the brain or other internal organs occurs rarely if ever. However, it is important to realize that the Parry Romberg syndrome of progressive facial hemiatrophy can look like linear scleroderma en coupe de sabre but does sometimes involves the brain. Parry Romberg syndrome most often can be distinguished because it will involve all of the face on the affected side and there is often asymmetry of the tongue.

If your child has isolated linear scleroderma en coupe de sabre, the cosmetic effect is your major concern. Since the lesion is easily seen, both you and your child will have to get used to people staring, or asking about it. Eventually you will discover that this has become a major irritant to both of you. No one has discovered a perfect way to deal with this. Three things should be remembered:  

  • Curiosity is natural – even if rude.
  • You have no obligation to give anyone other than your doctor an accurate medical description of the problem.
  • If you act ashamed of your child’s appearance, they will be ashamed of it.

I encourage my patients to think up the silliest explanation they can. Then I tell them to rehearse it repeatedly so they are always prepared to repeat it. Mothers can do the same thing. “I was burned there when my spaceship crashed... I was riding shotgun for Evil when he crashed on his attempt to cross the Atlantic on a motorcycle... An alien from a pink Cadiallac UFO touched me there one night...” The more ridiculous you make the story the clearer it will be that you are politely telling them to “bug off.” Not everyone will get the hint, but most will. Make up whatever silly story you are comfortable using. When you and your child have practiced a hundred times you can sneak smiles at each other and laugh at how shocked or gullible people are when you tell it. By making these events a source of amusement instead of shamed embarrassment you can markedly diminish the psychological trauma being obviously different brings.

Occasionally I see children who don’t fit the well described disease patterns. I’ve seen children with linear scleroderma en coupe de sabre who also have areas of involvement on their back or legs. Rare children with linear scleroderma en coupe de sabre have inflammation of the eye (uveitis) on the affected side. An opthalmologist can detect this and every child should have a periodic eye exam. Other problems have been described in one child in various reports, but it is always hard to know if they are really related to the linear scleroderma.

Many parents ask whether linear scleroderma en coupe de sabre could evolve into full fledged progressive systemic sclerosis. To the best of my knowledge there has never been such a case. That doesn’t mean I can promise there never has been or will be, but it does mean it is so unlikely that you shouldn’t be concerned about it.

Most children with linear scleroderma en coupe de sabre need little or no medical intervention. If the lesion is stable, it is unlikely that anything should be done. Cosmetic surgery should be considered only in the more severe cases. If you do consider it, make sure you choose a surgeon with lots of experience with linear scleroderma. These children do not heal well. At the hands of a plastic surgeon who is inexperienced or unprepared for the difficulties of linear scleroderma that results of surgery may be worse than the problem was.

When I see a child with rapidly expanding lesion on the face I recommend methotrexate therapy. Not every physician agrees with this. In many instances I have seen the lesions immediately stop spreading. Methotrexate will not undo areas of damage that already exist, and it is not without risks. If your child’s lesions are still expanding you’ll need to discuss all your options with your doctor. Most often the lesions have stopped spreading by the time the diagnosis is made and the child is seen by an appropriate specialist.

Children with linear scleroderma en coupe de sabre will need you to help them realize that they aren’t truly different. Over time they may also need the help of someone outside the family. This could be a physician, a priest, a teacher, a psychologist/psychiatrist, or any other warm supportive individual. With proper support and medical care they should live long, healthy, and productive lives just like everyone else.

Please keep in mind, this webpage is for your information only.
Please check with your child's physician for any treatments.

For more information on Juvenile Scleroderma, contact:

Juvenile Scleroderma Network, Inc.
1204 W. 13th Street, San Pedro, CA 90731

Tel: (310)519-9511 (Pacific Time)
24 Hour Support Line: 1-866-338-5892 (toll-free)

Speak to another JSD parent for emotional and logistical support provided by home-based JSD volunteers. For medical advice, please contact your child's physician.

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