June is scleroderma awareness month so I thought I would put together some information about scleroderma for you. First, it is important to note that scleroderma is an umbrella term for a group of diseases. I have been diagnosed with limited cutaneous systemic sclerosis so my blog tends to focus on that. If you would like to add to this blog with similar articles for other varieties of scleroderma or even for other autoimmune diseases or chronic conditions please drop me a line using my contact form. I wish I could pay you but this is a personal blog with no advertising to support it. I am not getting paid for my own work but am happy to do it to assist others who may be going through the frustration of dealing with chronic pain and illness.
I originally found these pictures posted on a Facebook friend’s personal website. They reminded me that I really should do something for Scleroderma Awareness Month.
As I mentioned earlier, I have the limited cutaneous systemic sclerosis variant of scleroderma so that is my focus here. This variant used to be called CREST based on the main symptoms of Calcinosis, Raynaud’s, Esophageal Dysfunction, Sclerodactyly, and Telangiectasia. It was changed to the current name limited cutaneous systemic sclerosis when they realized that this variant was systemic and could affect every body system including your organs, entire digestive system, muscles, joints, and even your blood vessels.
I’ve had a few doctors, including specialists, who think that limited means it is not too bad. I’ve also had doctors who didn’t believe me because I didn’t have any major skin changes. For decades I only had a small sliver of hard shiny skin right below my fingernails, now the tight shiny skin extends to my second knuckle. I also have it on my feet. Limited cutaneous means limited skin involvement. The different between limited and diffuse is that limited does progress slower than diffuse and diffuse has wide spread skin involvement. Both are systemic and both can affect everything in your body.
Calcinosis are deposits of calcium that start on the inside of your body near the bone. They can be extremely painful as they work their way to push through the surface. They can cause these horrible looking digital ulcers. Typically they seem to be on the fingers but they can happen anywhere. I have them in my feet as seen on x-rays. So far, I’ve been lucky. While it is difficult to walk due to pain, they have not caused ulcers.
Ulcers can get so bad that gangrene and tissue death can set in requiring amputation.
Raynaud’s constricts the blood vessels to the extremities. In some patient’s only the fingers and toes turn white but in others they can turn blue or purple with exposure to cold or stress.
This can be very painful and if you can’t get the circulation moving again gangrene can set in. My hands and feet will go white and a dark blue jean blue. I used to get asked if I’d spilled ink.
They will turn red when they start to warm up with pain and tingling.
The esophagus (simply put) is the tube that uses a wave-like motion to take your food from your mouth to your stomach. This picture shows decreased salivary production which causes dry mouth and tooth decay no matter how well you look after your teeth. It can also dry your throat making it even more difficult to swallow. If you have Sjogren’s on top of that swallowing becomes even more difficult.
GERD is very common with scleroderma causing acid reflux and heartburn. I don’t like to eat out any more because I am always afraid that I will choke on food that refuses to go down. It happens quite frequently. I recently had my esophagus dilated and food is going down better for now but only if it doesn’t get stuck in my throat first.
Gastroparesis is another dysmotility disorder. In a nutshell it means that your stomach does not empty well. This can get severe enough that you feel full on just a mouthful or two. Vomiting can occur and if it get severe enough a feeding tube is required.
Then there is dysmotility of the intestines. This can lead to severe bloating, constipation, diarrhea, and even obstructions requiring surgery.
Sclerodactyly is the term used when the skin starts getting tight and shiny. In severe cases the hands can become claw-like and very difficult to use. In Diffuse the entire body can be affected. With limited skin involvement is limited to the hands (up to the elbows) and feet (up to the knees).
There can be other skin involvement as well. For instance my skin in “mottled” on my chest and extremities. When I went to a cancer clinic after having a melanoma removed the doctor told me I had skin damage from spending too much time in the sun. I told him that I hadn’t sat in the sun for decades because it made me sick. He didn’t believe me pointing out the colour of my skin as his “proof.” When going over some old medical files at a later date I discovered that there was a name for the discoloration… Livedo Reticularis and in my case, it had nothing to do with the sun and everything to do with my autoimmune diseases.
Telangiectasia can appear pretty much anywhere on your body including your eyes. They are broken blood vessels. They can show as red dots like the photo or as red spider webs. I have them all over.
Muscle & Joint Pain
Wide spread chronic pain is a given with scleroderma and most other autoimmune diseases. A really good day for me would be like how you feel when you are running for Advil to help with the aches and pains you get from having a fever with the flu. I hate the question “How is your pain on a scale of 1 to 10?” because too many doctors don’t get my pain scale. When healthy people say a 10, I would be saying a 3. I am vomiting somewhere between 6 and 7 and am totally incoherent and don’t know where I am at 9.
Systemic forms of scleroderma can cause organ damage. I have PBC or Primary Biliary Cholangitis which is a liver disease that causes high blood levels of alkaline phosphorous. In PBC the bile ducts in the liver become blocked with fibrosis. It is NOT caused by alcohol. It causes liver pain and can eventually lead to requiring a liver transplant if it is not controlled.
As mentioned earlier, I have ILD or Interstitial Lung Disease which is now well controlled with medication but I am being evaluated for PAH or Pulmonary Arterial Hypertension where the pulmonary artery becomes enlarged and the blood pressure in the artery is high.
Heart damage can also occur. I was diagnosed with a “stiff” heart and told that my heart is strong but doesn’t empty the blood properly. Recently some other changes showed on an echogram so I have to go back to the cardiologist.
These are just a few of the things that can go wrong with your body when you have systemic scleroderma. For additional details be sure to follow the links below. I recommend the Scleroderma Education Project as a good place to start because it is an easier read.
The Scleroderma Education Project
I wanted to make sure that I mentioned the Scleroderma Education Project website since it is filled with excellent information for patients that have scleroderma. I love that it explains the different forms of scleroderma and provides updated information in words that are easy to understand. Ed Harris is the founder of this project, has limited cutaneous systemic sclerosis himself, promotes research, and runs a Facebook support group.
Please beware of using Dr. Google. There are so many seemingly legitimate websites that have dated and misleading information out there. On many occasions I have seen posts from patients who are terrified that they don’t have long to live. This may have been true years ago but there are new treatments available and current testing means that dangerous complications are caught much earlier giving a much better prognosis. As an example, I was diagnosed with ILD (Interstitial Lung Disease) a few years ago. It was caught in the early stage and treated. While I will take medication the rest of my life to protect my lungs the medication actually cleared up my lungs to the point that only some scarring shows on my lung CT now while previously they were covered in white spots.
Along with the Scleroderma Education Project some other websites you can use to educate yourself are The National Scleroderma Foundation, John Hopkins Scleroderma Centre, Cleveland Clinic, and some other major hospital websites in the US. I’m still looking for Canadian resources but so far all I’ve really found is Scleroderma Canada.