Erythromelalgia -- red, swollen, burning feet and hands

I know we've discussed erythromelalgia before, but I wanted to ask again how many of you have it. It came to mind because now that summer has arrived in full force here, I am feeling a lot better in general. I have far less pain, my skin feels better, I'm less stiff, my neuropathy is better (less tingling/numbness/burning), and of course the Raynaud's has calmed down a lot. So that's all wonderful! I hope the hot weather is making all of you feel a bit better too.

The one thing that is difficult about the summer is that I have erythromelalgia. My feet, and to a lesser degree my hands, swell and turn bright lobster red, burn and itch. This happens in hot weather, but also sometimes in air-conditioned places or even during the winter if I am standing for a while or while I'm walking. It also always happens when I take a shower unless it's ice cold, but anyone with Raynaud's knows that that would not be a good idea! When I get out of a warm shower my feet are usually so dark red that they look purple-black and quite swollen. Not very attractive! My veins (especially on the backs of my hands) get puffy and itchy and irritated when this happens and I sometimes get widespread itching and skin irritation.

I've had this issue for at least a few years (5-10), but I didn't know that it was an actual condition until recently -- I just thought I got overheated more easily than other people and didn't realize that it wasn't normal to have such an extreme response.

We've probably all heard that Raynaud's is a fairly common condition. Scleroderma is a rare disease. Erythromelalgia is even more rare. Supposedly it is almost unheard of for a person to have both Raynaud's and EM, and EM has never been positively associated with scleroderma in the way that Raynaud's has. So what I'm really curious about is how many of you have all 3 of these conditions. I'm also curious to hear if you DON'T have Erythromelalgia. I think it might be underdiagnosed in scleroderma patients because we just assume that swelling and redness and pain are part of the disease and don't present our doctors with these symptoms to consider independently.

I just read an article that some of you might find interesting. It is about the connection between EM and small fiber neuropathy. The article is asking whether EM is primarily a neuropathy or a vasculopathy. The researchers can't reach a conclusion, but they do note that people with EM are likely to have small fiber neuropathy (I do as well). http://archderm.jamanetwork.com/article.aspx?articleid=479530

Finally, if you have EM I'd love to hear any tips or tricks for dealing with it! My feet generally feel better after I elevate them. I'm wary of using anything cold to reduce swelling or pain because of the risk of Raynaud's, but I have sometimes run my hands under cool water to soothe them. Also, does anyone know if EM is progressive? I know that it generally doesn't improve and is pretty resistant to treatment, but does it always get worse? I've read stuff about people with EM being confined to bed or a wheelchair because they can't use their feet and of course this is a frightening thought.

Zoe

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I looked it up and also some pictures. It looks like something I have part of the time. Does it also really itch? Mine does.

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Hi Zoe, I have not been diagnosed with EM but am quite certain I have it. I thought it was just an unusual heat response until recently, so I haven't mentioned it to my scleroderma specialist yet. When on the beach I can suffer from my raynauds attacks if I go in the water and from cool breezes, or I can suffer horrible swelling/redness/burning/itching of my hands and feet from the warmth of the sun. My hands and feet swell, turn red, burn and itch every time I drink a warm drink as well. It also happens if I have to stand for too long. I think I will mention it at my next scleroderma visit. This is an interesting link from the "Raynauds and Scleroderma Association" site about EM: http://www.raynauds.org.uk/index.php?option=com_content&view=article&id=99& Itemid=208

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I have had numerous flare-ups undaignosised b/c I never thought to mention it since it is not painful. However in reading and seeing the pics, I think I have all 3 ; SSc, raynaunds and EM. I am on numerous meds that the one article list may help. I also have foot ulcers so I have had to elevate them alot of times anymore which helps to eliminate the EM reactions.
One article list that it could effect internal organs, so my ? would be is that why the heat really excrebates the a-typical asthma???

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Yes, my feet and hands get very itchy in the heat. Actually I kind of get itchy all over! Some people with EM just have burning pain, but I've read that itching is common too.

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It definitely sounds like you have it too. I suspect that a lot of people with scleroderma do, but they aren't being diagnosed because they just accept it as another weird symptom of their disease. If you were perfectly healthy and suddenly developed swollen, burning, itching, red feet and hands, then you'd probably ask your doctor. But if you already have lots of bizarre and unpleasant symptoms, you probably just shrug your shoulders and cope the best you can!

I hope that people who read this and suspect they have EM will mention it to their rheumatologist (or neurologist if they have one) because it seems like we all have a lot of symptoms in common that doctors don't recognize as being part of the disease. EM is extremely rare so you would not expect so many of us to have it if it were simply a coincidence. I think I read that something like 400 people in the US currently have EM. Or was it 4000? In any case, a very small number.

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I do.

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I have EM on the tops of my feet (not the bottoms), but never on my hands. It gets worse in the evening. Sometimes only ONE foot gets red - it can be either one. The only remedy I've found is to raise the feet and keep them cool. I also have small fiber neuropathy and dry, itchy skin, but not Raynaud's.

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I have schleroderm raynauds and erythromelalgia
My schl symptons are mild but the raynauds and em are the problem
I have had em for five years and take Pregabalin and Cymbalta
I have just started on an ace inhibitor Ramipril but if anything itsd making my em worse nad not heloing the raunauds
as its just been a week since i started i am giving it another week
I take oxycontin for oain slow release and stat doses
It helps
There is no cure for em
To cure the raynauds uncreases the em so its a catch 22
Im house bound
I can barely walk for two minutes before im in agony!
Nothing really gets rid of the pain but the oxy reduces it down from a ten plus to a six or a seven
Its a case of management

If new with em please do not soak your feet in water - it will only make the em owrse even though you think its giving you short term releif
go stand on a cold floor or similar
Dont use creams or lotions of any sort on your feet
keep a constant room temoperature where possible
dont wear shoes or socks etc
if em not too bad get sock made from bamboo

join The erythromelalgia assosciation cant spelkl it ! association
plenty of articles about em

the problem wiht raynauds and em is of course finding the middle ground where feet not too hot or too coild
Im still trying

if blue with cold and try to heat up i end up with a flare up of em
these flares can go on for days and weeks at a time with no relief even with the oxy
ive tried everything under the sun as a form of pain relief apart from iv ketamine

Ive had consultants scratch their heads not knowing a thing about em
Ive been patronised , talked to like im an idiot and sent away with drugs drugs and more drugs

Dont be suprised if your doctor doesnt have a clue
Do your own research
get data from the TEA

Be your own advocat becasue theres so little knowledge within the medical workd you have to be

There is research going on

Pharmeceutical companies are doing research 0 they want to be the first to find a pain killer that will deal with pain such as em pain as they will make millions if they do

Is it a neuropathy or an arterialopathy

Who knows!
one of the pain doctors ive recently seen is convinced its not a neuropathy and dismissed my saying ive got small fibre neuropathy
but then i didnt lkike him one bit he was talking to me as if i was a complete fool and just someone else with pain,,,,,,,

There isnt much i dont know about em

If not taking already try the lyrica /cymbalta combination

But everyone responds differently
becasue the SCN9 gene has many sub stratas and the Nav 1.7 isnt the only culprit for casuing pain and so we may all have differing malfunctions of SCN9 gene and NAV1.3 through to NAV 1.18

Its isolating its agony and its perplexing and painful

the focus has to be on pain management

most only respond to narcitics /opoiates such as morphine or oxycontin

anything else doesnt touch the sides

I belong to several groups re em and gain knowledge everyday from others input so its worth joining nay group you can find
my favourite group is a yahoo group for erthromelalgia

amanda

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i read that you have ulcers - please please do not soak your feet in water to cool them
It will make the ulcers worse as the water casuses celular damage and exacerbates the breakdown of the tisse and increases the iulcers

If you can tolerate it you should look at some form of compression stocking - talk to your doctor

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i know you say you get relief with badger balm but in truth it is most likely to actually perpatuate the em
I understand its soothing but it is likely to cause the blood vessels in your feet to remain dilated
Yur better off standing on a cold surface
I use a block of marble which works well

There is no cure for em its a case of managing the condition

Ive has EM ( with schleroderma and now raynauds ) fir iver five years and have researched the condition to death ive also tried and tested almost every known "treatment" and if i javent tried it i know another em sufferer who has!!

Please make sure you dont soak your feet in water either - this causes tissue break down and will lead to ulceration over time

No socks ! No stockings! open sandels
some can tolerate socks made from bamboo

if the pain is very bad you could try Lyrica ( pregabalin )

if worse than very bad, its usually only an opiate drug that wiull work becasue of the nature of the pain
I take oxycontin with reasonable effect as well as Lyrica and Cymbalta

Hope this helps
Amanda

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The only time I have had any symptoms like EM is when I had a bad case of Raynauds because I stayed out in the cold weather too long. I made the mistake early on in the condition of trying to warm my blue fingers up too quickly in water that was too warm. I then had the red, swolen and itchy hands. I got an education real quick not to do this again. I hope I don't develop this condition (EM). There are so many of the other symptoms of limited systemic scleroderma that I have. Pulmonary Fibrosis is the worst.

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OMG!!!! I just thought this was the way my feet were. I had absolutely no idea there was a "name" for it. My feet are red, they burn, they itch and they only relief I can get is to stand on the hardwood floors. As crazy as it sounds, I have often told my husband if he would get me a bucket of ice I would gladly submerge my feet in it!! And if I ever did it I would guarantee that steam would rise because my feet are so hot while they feel this way. I am fairly recently diagnosed with morphea scleroderma and it is present in my feet so this is all now making sense. I have an appt at Vanderbilt in a few weeks and I will bring this up. Thank you so much zoe123 for the post. You may have just helped my husband realize that I am not crazy!!!
Have a great day all!!

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Glad you might have found a name for something that's bothering you! That's half the battle. ;)

Unfortunately, there's no cure for it and treatments are hit-or-miss, but at least there may be some things you can try. It's a neurovascular disease, so either a neurologist or a vascular specialist is most likely to be able to help. Since EM is so rare, most other doctors might not have even heard of it.

Stay away from the ice! I know you were joking, but watch out for cooling your feet down too much. If your feet are bothering you a lot you can use a fan to cool them down or run some tepid water over them, but don't go for cold water or ice because you can end up causing damage.

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Hi Zoe - you sound well informed about em which is a rarity in itself! I live in the UK and know about 12 people with em Im sure there are more. All of us have primary em ( ideopathic) and are female and over 40. Several of us have em and Raynauds. I seem to be the only one with a diagnosis of schleroderm, This came 5 years after em and at the same time as the raynauds diagnosis, Mind you it was retty easy to identify .
The em is the greater issue for me. I recently saw a pain sopecialist attached to Cambridge university and he was convinced it was vaasculopathy secondary to schleroderma with raynauds and started me on an ACE inhibitor Ramipril. Well....... never again,
I forced myself to take it sepsite my fears that as it was a vaso dilator it would make my em worse and boy did it, I took it for seven days and on the seventh day i experienced pain beyond any pain scale. It was the owrst flare up i have ever experienced and large odses of oxycontin and morphine did nothing but reduce the pain by about 10 %......nothing! this flareup has continued now for 24 hours and is driving me crazy as you can imagine

I will never ever touch an ACE inhibitor again

So a word of warning to anyone who is offered this category of drug... be warned!

I was a registered nurse and have a reasonably good level of knowledge and have made it my mission to know all there is to know about em and raynauds
My knowledge of schlerodera is less, I was diagnosed with limited sch but I read alot of confliting data about how this can progeess or not

I cant type to save my life and am a dit dyslexic so forgive all the tpyos!

Anyone got any clinical papers re limited schleroderma that the ccan refer me to?

Many thanks
Amanda

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Hi Amanda,

It makes sense that the Raynaud's and EM are a result of vasculopathy related to the scleroderma. I often think that at the most basic level my problem is a vascular one and that the vascular damage is causing all sorts of other issues including pain, migraines, neuropathy, and skin issues.

I tried another type of vasodilator, a calcium channel blocker called Amlodipine/Norvasc for about 6 months. It neither helped my Raynaud's nor worsened my EM! However, it was a very low dose because I have low blood pressure and didn't want to risk lowering it further and increasing dizziness or causing fainting (something that happens on occasion anyway!)

You'll find A LOT of information about limited scleroderma on this board. You can search PubMed for recent articles, but there will be many to wade through. If you want information about a specific topic related to scleroderma, try posting a discussion question on the board and I'm sure you'll get a lot of great replies from people who have experienced the exact thing you are wondering about. Are you most interested in hearing about prognosis? It seems highly variable with some people experiencing serious complications (lung, heart, kidneys, GI) within the first few years, while many others go for years and even decades with only mild symptoms. In general, limited scleroderma is a less serious disease than diffuse scleroderma with slower progression and less severe internal organ complications. Unfortunately some people with limited skin involvement do end up getting life-threatening organ damage. People with limited scleroderma are particularly at risk for pulmonary hypertension which tends to develop after many years (or decades) of living with the disease. You will also find people who live in relative comfort with limited scleroderma for 20, 30, or 40 years and eventually die of something else entirely like the more common ailments of cardiovascular disease or cancer.

If you are looking up statistics and information about prognosis, please look only at recently published articles and studies because treatments have improved a great deal. For example, kidney involvement used to be fatal and is now highly treatable. There have been advances in treating pulmonary hypertension recently. Stem cell transplants are being investigated and look very promising. There was recently a very interesting discovery made by a scleroderma researcher that may eventually be developed into a therapy to stop or reverse fibrosis (though the actual drug therapy is probably a long way down the pipeline).

Zoe

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All interesting replies. I have learned so much on this site that validated the symptoms and pains that I have. I thought I was crazy or just overreacting. Before I was diagnosed with CREST I first experienced my hands being on fire and turning red. This happened a lot after eating something hot like soup or when I ate anything if I waited to long before eating my first meal of the day. Then the raynaulds started and it was years after that that I was diagnosed with CREST. After the diagnosis slowly things got worse for me. I used to water ski everyday and snow ski but my feet began to hurt, excrutiating pain, that I had to give up water skiing completely and most snow skiing. Then just walking on my feet became unbearable some times, especially in the morning. Now my feet are hot and red on the soles. I dont wear socks and I do not put my feet under the covers at night. I did not think much about my feet being hot, I would just put more lotion on them, the skin is very dry and cracks. then I saw Daisy Do's picture and when I looked at the sole of my feet tonight they were beet red. It is nice to have these things validated, most doctors dont know very much I think since it is so rare they probably get very many patients to compare and just learn from their textbooks.

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I'm weighing in for your casual survey. Swollen, itchy, burning, sore, lobster red feet? You bet I have this, along with Scleroderma and Raynaud's. I don't experience this with my hands, though. The swelling becomes so drastic in my toes that some of them eventually burst at the ends and then I have painful, weeping, open sores. Usually happens for me during the warm months.

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I complained about my feet and not being able to walk and my PC sent me to a foot Dr who wanted to sell me $200.00 shoes and said that was my problem...the thought was too painful to consider, so I continued to hobble in cushioned slippers. My Dermatologist, gave the blank look, no help there.......You can't show it unless it is happening, Daisy-do's photo says it all...as soon as I saw it I knew what was the matter. So I took a similar picture of my feet, showed it to my Sclero Specialist, and got the same blank stare......he didn't appreciate the self diagnosis. On my own I found relief by elevating and put a fan on them. and over time the pain does get better for me. I have used "Sore no more" cool therapy cream for some temp relief. we have to do what works for us? Sure does help to have this forum !! good luck to all

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I have mixed connective tissue disease with symptoms of three of the four overlapping diseases you can have symptoms from. I had talked to so many doctors for years about my feet and sometimes my hand and ended up finding information regarding em on my own on the internet of course. It happens anytime I stand still for a few minutes, it drives me crazy, it's like an intense itching or crawling sensation in my foot and ankles and every little blood vessel pops out on my feet and the soles of my feet turn bright red. I have raynaud's as well, and my doctor also said something about reverse Raynauds? Has anybody ever heard of that? Maybe he was just saying that's what it's like, because it is. When cold the vessels constrict and when hot they dilate. I just assume it's part of the scleroderma and vein issues. I am lucky at this point because I can control how long I stand still most of the time and make it go away. I'm sorry for those of you who suffer with so much pain from it!!

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Yes, I think your doctor meant erythromelalgia when he said "reverse Raynaud's". The funny thing is that I came across articles stating that erythromelalgia is so incredibly rare (more rare than scleroderma) and it is almost unheard of to have both EM and Raynaud's in the same person! Obviously people with connective tissue diseases can and do have both conditions more often than doctors recognize. I have NEVER read anything about erythromelalgia being a symptom of, or related to, scleroderma (or MCTD). But I imagine that many of us would fulfill the criteria for diagnosing it.

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