Tess: Scleroderma En Coup de Sabre

Hi my name is Tess. I am thirty years old and I have just had reconstructive surgery for en coup de sabre, which is a form of linear scleroderma.

Mine started with a bruise on my forehead at age four that continued to grow until it covered my left eye and moved in a straight line up to my hairline. By the time we saw a specialist the indentation had begun. He told my mum it was a sabre cut and nothing could be done until it finished and I had stopped growing. That was it!!

The indentation started right on top of my left eye and continued its course up my forehead and right through to the back of my skull (crown). I had dark pigment along with it which made it look worse and my skin was so thin you could see my veins and nerves even through the pigment.

I was lucky enough that my hair could cover it and that turned into an obsession. I would have anxiety about windy days blowing my hair and I was worse when it rained as it would make my hair a complete mess. I never learned to swim for fear of my hair getting wet and rarely went to hairdressers for fear of people staring and asking questions to which I had no answers. (I love the person who invented hair spray.)

School was terrible with constant teasing that made me want to crawl under a rock and not come out. I got called "dent head", "panel beater", "grand canyon", "polly filler" and more. I became very defensive, aggressive and emotional which were a lot of mixed feelings for a child to control (even as a teenager). I had a lot of great friends though who did their best to stand up for me even though they knew less about it than I did about it.

When I was twenty-three, I was working night shift in a factory that made me very run down and tired resulting in my immune system going silly. I caught every cold, flu and infection that was going around and it seemed like I was never healthy.

Then came the alopecia areata! Wow! Another horrible thing to deal with, and of course my hair fell out all around my "dent" through to my crown and I even lost my left eyebrow. My hair obsession became worse then as nothing I did could cover all my loss. Eventually it grew back, but I still now suffer with small patches occurring all over. When one patch starts to grow back, I find another. My once very thick hair is now a lot thinner.

Three years ago I found an article in a magazine about Parry Romberg Syndrome and the girl looked liked me but without the dark pigment. I had never seen anyone else like me before. She told of her reconstructive surgery using her own fat and after I got over the fear of her surgery, I got a referral to the cranial facial unit.

They told me it was scleroderma (nothing else) and put me through six months of blood tests, X-rays, CT scans and more, and put me on a public patient waiting list for surgery. I did find out that the scleroderma has finished its destruction, hopefully.

I am on my fourth day of recovery from surgery in which they cut a solid mass of fat (with skin layers) from my abdomen. Fat injections would not work as they needed more skin due to the thinning. They molded this into my "dent". They cut off the dark pigment and stitched the two sides together. I should have a hopefully thin scar and my eyebrow will be shorter.

I am a big baby when it comes to pain, but to me this pain from surgery is worth it. It will probably be another year before the scar finishes healing properly. I have not just done the surgery for look's sake, but for my own confidence and to feel whole. Hopefully I can break my long-standing hair obsession too!

I am only beginning my journey of learning about scleroderma and have just found out through the Internet that this is a disease and there are so many different ones affecting people in other ways. I have not found anyone yet with en coup de sabre, although I have lot more searches to do. It is very much an education reading peoples stories, so that is why I wanted to share mine.