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Linear Morphea/Coup de Sabre


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#1 celzsing

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Posted 22 September 2009 - 02:38 PM

Hi,
I just today received the diagnosis of linear morphea with scleroderma and coup de sabre. I actually went to the doctor today to report a suspicious mark on my skin, which indeed was a keratosis (pre-cancer) and was removed. While in the office, I told the doctor about my other problems and he miraculously knew exactly what it was! It was amazing to get a diagnosis after 10 years of suffering and 15 since the first sign. He said it was very rare and then proceeds to show me off to his colleagues. He really made me think it was no big deal, sent me home with some vitamin D ointment and I thought all was well. Now, I'm looking online, my mom (a nurse) is talking to colleagues who are doctors and I'm realizing there is no clear course of treatment, very limited studies and statistics and no definitive “cure!”

When I was around 12 I first noticed a long, brownish-pigmented line running down the center of my forehead, which was relatively light and would darken in the sun. I went to a dermatologist and he diagnosed it as a "cafe au lait" mark and offered skin-bleaching creams. My mother and I declined this offer. When I was about 17 (I am now 27), I began having a small bald patch on my scalp, near the front of my hairline. It was about the size of a dime when I first reported this symptom to a doctor. (It is now about 5 inches long!!!!) I was told that it was stress related alopecia and was given a cream, which I now forget the name of, possibly dovonex. I used this cream and still the symptoms worsened gradually over time. I later noticed that my scalp had become severely indented with a ridge on the top center and a wide flat slope at the linear bald patch, characterized by scarring. There is also a linear indent next to the original brown line down the center of my forehead. About 3 years ago, I went to another dermatologist and was diagnosed as having scleroderma. I was given cortisone injections on the site and was treated only for the bald, scarring area. I was told that he (the dermatologist) had never seen anything like it before and was not given a definite diagnosis. At the time I saw this doctor, the area was red, enflamed and had a tingling sensation, which I now knew meant it was "active". The cortisone injections stopped the progress of the symptoms temporarily and I was again given a cream, this time I am sure it was Dovonex.
Eventually, the symptoms returned, I became frustrated and just accepted this unwelcome menace to my anatomy.

In the past two years, I have had aggressive, chronic migraines and stomach problems (GERD). I also have been blessed (sarcasm) with a brand new coup de sabre ("mark of the sword" what looks like a linear dent with brown markings) across my forehead that has formed in just the last six months! I have no idea where to start and I can tell that my disease is becoming more aggressive. Please, if you know anything about this, let me know.

#2 janey

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Posted 22 September 2009 - 03:29 PM

Celzsing,
Welcome to the Sclero forums! I'm so sorry to read about your current diagnosis and years of dealing with doctors that can't recognize what you have. I have systemic scleroderma so I can't provide any personal experience or advice on the types to sclero that you have. However, we do have several pages on linear scleroderma and a page on En coup de sabre. I think you'll find a lot of information as well as links to stories written by people who have these forms of scleroderma.

Have you seen a scleroderma specialist? You really should if you haven't. I'm no doctor, but I do believe there is more you can do than just rub a topical cream on your skin. Please check out the link provided.

Again - I'm so glad you have joined us. I'm sure you'll hear from others. Please keep us up on your progress.

Big Hugs,
Janey Willis
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#3 Amanda Thorpe

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Posted 23 September 2009 - 07:41 AM

Hello Celzsing

Welcome to the forum! From your post I gather you have been diagnosed with localised scleroderma which includes linear and en coup de sabre. The down side is that with all forms of scleroderma there is no cure for localised but the up side is that there are treatments that may help the symptoms. As Janey has already suggested it may be worth your while to see a scleroderma axpert so that you can get a treatment plan underway. Here is a link to worldwide speacialistsif you want to have a look.

I have systemic sclerosis as well as morphea (the other form of localised scleroderma)so I have the best of both worlds! :VeryHappy:

Take care and keep posting.
Amanda Thorpe
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#4 Shelley Ensz

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Posted 24 September 2009 - 01:58 AM

Hi Celzsing,

Welcome to Sclero Forums! I'm sorry it is because you have localized scleroderma -- and such a delayed diagnosis, too.

There are treatments available for en coup de sabre now, and it is something you'd particularly want to discuss with a scleroderma expert.

I'm very sorry it took you so long to get a good diagnosis.
Warm Hugs,

Shelley Ensz
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#5 Carol Lee

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Posted 24 September 2009 - 10:21 AM

Hi Celzsing,

I have linear scleroderma on my scalp. It started when I was about 17, I am now 57 so I have had it 40 years! It started as a small area on the top of head was red inflamed and sensitive, with hair loss. The dermatologist then just told me to not to pick at it, but I was not picking at it. The area grew to about 2 1/2 inches long on the center top on my scalp. When I was 29 my right ankle became swollen for no apparent reason. Over the next few years the swelling in my ankle spread to my entire leg from my toes to my groin, was diagnosed as lymphedema, but could find no cause. I found a great dermatologist when I was 30. He had me go to plastic surgeon to have the area on my scalp removed. After the surgery the area healed well and looked great, but then 6 months later I started to loose hair in the area and it came back the same exact area as before! He treated it with cortisone cream but it did not have any effect. This doctor was connected with the university and a teaching doctor, he found a great surgeon for my leg that did surgery that help relieve the lymphedema. My dermatologist also liked to bring in students to look at me. This was happening when there was no computer and so I could not get very much info on scleoderma. My dermatologist said the swelling of my leg was caused by scleroderma. Over the years the area on my scalp has slowly progressed to 4" and the bald area are not so easily covered by the remaining hair. My dermatologist has been retired so I saw a local dermatologist several years ago. This dermatologist said he has been in practice for 30 years and had never seen linear scleroderma before, he took a photo of it and gave me dovonix. The dovonix irritated the area so I couldn't use it. The area continues to slowly progress. I thought it would be great to see scleroderma specialist since I also have upper gasto intestinal problems, hypothyroid and chronic fatigue. When I went to specialist he did not offer any kind of help. When I told him the area on my scalp is progressing he said no it is not. That just floored me. He also said that he didn't think the lymphedema in my leg was caused by scleroderma and that biopsy can not diagnose scleroderma! Frustration after all these years and no answers. So now I don't know if there is any treatment that can stop the progression, probably not, but it would be great to be able to find some treatment for it.
Carol Lee

#6 Jen

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Posted 24 September 2009 - 06:48 PM

Hoping to give you a little bit of hope here.

When I was 24, I started to notice that every month or so my scalp would start getting really oily. It came on very suddenly. I'd wake up with really oily hair and the only thing that stripped the oil out was dishwashing soap. This went on for about a year. Then, about the time I noticed a very small spot on my hairline that was bald, I began having very severe joint pain and inflammation. Doctors considered rhumatoid arthritis, lupus, and all sorts of things. The spot on my scalp began to increase and form the all-too-familiar line from my scalp through my forehead. Doctors were baffled. Within a few months, we knew what we were dealing with. Cortizone shots, topical ointments, heavy meds to decrease the joint inflammation... How frustrating.

I met and married my husband during this time imagining that I was entering us both selfishly into a life of doctors, meds and restrictions. It took a few years but I'm happy to report that it burned itself out. I went into remission. At 27 I was completely off meds, and "baby-hairs" were growing in the place of the line on my scalp. Though not all the hair grew back (have a small quarter-size scar and a small scar line on my forehead), everything else disappeared. Since then, I have greater respect for those dealing with chronic health issues, and a better perspective on pain and perseverance.

Since we are close by, last year I saw a Stanford specialist in dermatological autoimmune issues (couldn't believe there was one). He confirmed it was in remission and said it wasn't likely to return. I'm 35 now with a 4 year old son and I'm still reminded of that heavy season, and still thankful for it's brief stay.

I know that many of you who are newly diagnosed may feel like this is a lifetime disease, so I wanted to make sure to let you know that not every case is a lengthy one. Bless you all!!

#7 Amanda Thorpe

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Posted 25 September 2009 - 07:41 AM

Hello Carol Lee

I am so sorry you had such a frustrating encounter with a doctor supposed to be a scleroderma specialist. I can confirm that you can diagnose scleroderma with a biopsy, I know I had one!

We have a list on this site of bona fide scleroderma experts and it may be worth your while seeing one of them. Unfortunately we all seem to experience a difficult encounter with a doctor somewhere along the line, please don't let it stop you from getting the treatment you deserve.

Take care.
Amanda Thorpe
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#8 Amanda Thorpe

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Posted 25 September 2009 - 07:45 AM

Hello Jen

Welcome to the forum and I am so pleased that you have had a positive outome! It's great to know when someone has overcome scleroderma!

Take care.
Amanda Thorpe
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#9 Carol Lee

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Posted 26 September 2009 - 04:12 AM

Hi Amanda,

What is so surprising is that this doctor is a scleroderma expert and has been working with scleroderma patients for 30 years! So now I have 2 different opinions, both doctors experts. The sclero expert said that extra collagen seen in biopsy can be caused by other things, such as trauma. So even with biopsy he didn't think the lymphedema was caused by scleroderma. Well the lymphyedema in my leg appeared without a trauma, he did not say any other reasons for the extra collagen. This doctor also said that scleroderma is not systemic unless you have it in your hands, heart or lungs. He was very nice but not really helpful. When I saw him I was tired, it was 2hr drive and so surprised at what he said that I did not question why he said that the linear sclero was not progressing when he had not seen it before. He ask if I had seen the dermatologist so maybe he is saying he does't treat linear scleroderma. So I just have no answers for my problems. But my understanding is there is really no proven treatment for linear scleroderma and if even having surgery to remove it didn't work, I am not optimistic that it can be stopped. I was just hoping to get good some information from this doctor, but that didn't happen.

Carol Lee