SPOTLIGHT ON: Living with Erythromelalgia and Raynauds

Dear LWE Members,

As from June we will be featuring a monthly spotlight on EM related - allied conditions. Our first SPOTLIGHT ON is RAYNAUDS SYNDROME .

Are you living with EM and Raynauds ?

"Raynaud's phenomenon occurs when the extremities of the body, usually the fingers and toes, change colour and may become painful. It is due to a narrowing (constriction) of the small blood vessels on exposure to the cold, or to a change in temperature, or to emotional stress. In most cases, the cause is not known. In a minority of cases, it is a symptom of an underlying condition such as scleroderma, lupus, or rheumatoid arthritis .It frequently co exists in EM sufferers. The main treatment is to keep warm. Vasodilators are used in some cases".

We welcome your suggestions and ideas for future SPOTLIGHT features

246-RaynaudsTips.docx (14.3 KB)

Attachments

254-EMandraynauldssclerodemaassociation.pdf (936 KB) 255-MechanismsofRaynauddisease.pdf (240 KB)

yes I do have both.no medication has helped as taking it for one condition sets off the other.so i'm unfortunate in that sense but live in hope of some new treatment in the future x

I tried talking to my gp about Raynauds about a year ago. She said I didn’t have it because I don’t “blanch out”. Last week when I tried to talk to her about EM she said “no…you have Raynauds but you don’t seem to blanch out”. I know what my body does when it’s cold OR hot and I do believe I have Raynauds. I also believe I have EM because Raynauds doesn’t cause the red and hot (and sometimes purple) that I have. At least this is how I understand it. If there are those among us that have JUST Raynauds but can get hot like this please let me know?

Patricia, I doubt if there is anyone with just Raynauds who gets red AND hot. I had Raynauds for about 46 years before I had EM. My fingers would go absolutely white and then purple and red and would hurt a lot at every stage but no burning, no swelling and no heat. I think my Raynauds is pretty typical.

Now of course it is different as a Raynauds episode will be followed by the burning heat of EM which can keep on going for many hours.

As copperowl says treatment when you have both conditions is difficult. I was in clinic last week and they have asked me to consider Iloprost infusions. I am not sure I want to go down that route as it involves 5 days in hospital, the threat of headaches when I already have migraines, and people have reported that any improvement can be short lived.

Managing it is my preferred way of dealing with it, given that aspirin has worked to reduce the swelling and intensity of flares. Winter is difficult, always cold but scared to provoke a flare.
Raynauds also affects my toes, ears and nose. My nose didn’t blanch but just went red and stayed that way when I warmed up. I am retired now but when I was working in school a cheeky 10 year old asked me if I drank gin. I said no and she replied unnecessarily loudly “because my mum says it’s drinking gin that makes your nose red”. Thankfully I only had to visit that school once a week.

I just read the document Raynauds tips and it made me feel very uncomfortable. At least half of them would be a complete no-no for someone with EM. That is the whole problem of having both conditions. I can’t sleep under a duvet, wear multiple layers, heat the house more than the minimum etc etc. As for heating up my clothes - instant flare!

I have both. It's a constant see-saw for me, trying to find the balance between warming up and overheating. In order to cope, I have changed some basic habits and the list of "necessities" that I carry around with me. First of all, I dress in layers so that I can add on and remove as necessary to accommodate my back and forth temperature needs. I always carry around an extra sweater and pair of socks.

I live in Houston, Texas where it is extremely hot in the summer. Businesses tend to overcompensate for the heat outside by keeping their establishments very cold on the inside. I get lots of curious looks because I always seem to be the opposite of what everyone else is feeling... wearing sandals in the winter (because everyone has their heat cranked up inside) and sweaters in the summer (to offset the frigid indoor temperatures).

It is a challenge to stay comfortable and it often gets frustrating. At night, I do the whole "covers on, covers off" dance.

People are always curious and will ask. I found out long ago, that they can't really process what I'm telling them so I gave up trying to explain in detail. Now I just say that I have a condition that makes it hard to regulate my body temperature and leave it at that.

Clearly there are a lot of us who have both Raynauds and EM. Are you thinking that the EM is secondary to the Raynauds, or that one is caused by the other and which way round?



Nel said:

Clearly there are a lot of us who have both Raynauds and EM. Are you thinking that the EM is secondary to the Raynauds, or that one is caused by the other and which way round?
I was diagnosed with Raynaud's years ago-maybe 18 or so years. But I didn't have it flare much because I had moved to Fla. It has gotten worse as I lived in different climates. Dx. recently with EM, but looking back had symptoms for a few years.. Now both are so horrific I really wonder what has been happening for decades that was mild. This is all so confusing.

Twinkle toes, it is confusing and that’s why I wondered which came first for other members. I have seen people write that they had EM before Raynauds, others the opposite. In my case Raynauds came first by 46 years so no doubt there! However at the same time as EM began the Raynauds became much more of a problem.
It is puzzling indeed and I don’t know the answer and nor do any of the doctors I have seen.

DId EM result from the Raynauds? OR

Did EM result from another cause (exposure to chemicals, hypothyroidism, surgery, ciprofloxacin, some as yet undiagnosed condition for instance - I could make a very long list) and merely made the Raynauds worse.

Because we are putting Raynauds under the Spotlight this month I have been thinking a lot about it but normally I don’t bother myself. It is what it is and I have just got to live with it.

By the way kruizerchick, apart from where you live I could have written your post myself, word for word.

I was on Pentoxifilin for Raynaud's for about 2 years. I was also taking nipedifine for about 6 months. I have no idea if the Pentox did anything--I was on a lot of it too (can't remember the dosage but it was 3 pills a day). The nipedifine seemed to help the Raynaud's somewhat, but then summer hit and oh my god, my lower legs and ankles swelled like I'd never seen before. Insane. I went off the nipe, and the swelling went down a lot, then got off the Pentox, and it's pretty much gone. It's been like 3 weeks off both. My EM is really intense this summer. Hard to know why--maybe related to those meds, maybe just getting older, maybe other stressors. Heat, of course. I've been doing one aspirin a day for years. The main thing that saves me from ulcers in the winter is behavioral--just had to learn how to protect my toes, especially when biking. A lot of $$ invested in my "system" and really good socks. Of course, shoes and socks are confusing when you've got both Raynaud's and EM, so, yeah, just always trying to think ahead, taking alternative socks and shoes whenever I can.

From members previous post on Raynauds 2013

I’m posting this here cuz discussions are more read than blogs and gotta say I found this very interesting. Obviously my case. :slight_smile:

"It is interesting that most sufferers of erythromelalgia also
suffer from Raynaud’s. In Raynaud’s, the digits become

cold and painful due to a tightening or constriction of the

arteries which normally supply blood to the affected parts.

In erythromelalgia associated with Raynaud’s, the sufferer

does not necessarily notice the blanching of the digit or

limb but is merely aware of the hot burning extremity. It

would therefore appear strange that treatment for this

form of erythromelalgia is to dilate the blood vessels.

Superficially the symptoms of Raynaud’s and

erythromelalgia appear to be opposite i.e.

vasoconstriction in the case of Raynaud’s and

vasodilation in erythromelalgia. It has recently become

apparent that this may not be correct and the evidence for

increased blood flow as a cause of erythromelalgia is poor."

from: http://www.raynauds.org.uk/images/stories/75-em.pdf

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Replies to This Discussion

Permalink Reply by liz on February 4, 2013 at 4:13pm
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now I know why sympatectomy helped me. What a pity it had not permanent effect for my hands. But who cares & thanx God for the feet! Hopefuly they gonna stay this way :)

again from: http://www.raynauds.org.uk/images/stories/75-em.pdf

"

A mild vasodilator (to
prevent the original Raynaud’s phenomenon) or
analgesia can help some patients." (EM patients)

Permalink Reply by clarissajo on February 5, 2013 at 12:52pm
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In my lupus groups, especially in the lupus community on Ben's Friends, people tend to ignore me when I say that they should explore the possibilty of EM when they experience redness and burning pain in their extremities. I'm the only person who mentions EM, everyone else always attributes these symptoms to Raynaud's, "Oh, my Raynaud's is all different colors, white, blue, and red.... it's just Raynaud's".

I get frustrated with this attitude, and the way that people aggressively try to shut me down by completely ignoring me and making statements in direct opposition to the medical facts I post about EM. Does anyone else relate to my experiences with trying to raise awareness of EM in communities that have a high incidence of Raynaud's?

JoAnn

Reply by aftertheteacups on February 5, 2013 at 4:50pm
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Hi! Maybe it's just that I've had a long day at work and am having trouble focusing, but I don't quite understand what this passage is saying (I looked at the rest of the link too). Are they saying that EM and Raynaud's are not related to vasodilation/constriction? And if not, what are they proposing is the answer?

Thanks for posting the info, Liz!

Permalink Reply by In pain on February 6, 2013 at 12:37am
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I have both conditions

Permalink Reply by liz on February 6, 2013 at 7:34am
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So first to JoAnn, ppl never pay attention about anything until they dont have to deal with it on "their own skin". So, I'm not surprised about what you're saying of ppl on forums. :)

Now to my post. From my understanding, the article is saying that superficially EM and Raynaud's may appear like completely different (oposite) conditions. One caused by vasodilatation and other by vasoconstriction. BUT that in cases when ppl having BOTH conditions, they don't stand like two different things, but are related. Means the worse your Raynaud's is, the worse will your EM get, simply like if the body is trying to "fix" the extreme cold by making it hot, but it kinda overreacts. That in these cases when patiens dealing with both conditions (EM is the more annoying one, no doubt about that) it helps (in my case it definitely does help) to treat the Raynaud's as a primary condition by using vasodilatators and that it simultaneously improve the EM. As they wrote "A mild vasodilator (to
prevent the original Raynaud’s phenomenon) can help some patients." (patients with both conditions)

It sounds very logical to me, tbh I always felt it this way, so for me the words I read were just reassuring information that I understand by body well.

Simply, if you have Raynaud's, Erythromelalgia can be just secondary to it. If you treat Raynaud's your EM goes away.

That's why my sympatectomy surgery (which dilates the small vessels) has such a possitive effect, despite I was more bothered by EM than Raynaud's before. "In erythromelalgia associated with Raynaud’s, the sufferer
does not necessarily notice the blanching of the digit or
limb but is merely aware of the hot burning extremity."

Using vasodilatators is definitely helpful in these cases. Let's call it EM secondary to Raynaud's.

I hope I wrote this at least a lil bit understandable, I'm not native English speaker, so sorry for this. :)

This all also means when you have EM secondary to Raynauds that every cooling down your extremities during the EM flare makes it all only worse, putting you into the vicious circle. The more cold the more hot follows. :)

For me this works on 100%. And dont forget it doesnt have to be just getting really cold and feeling really bothered by Raynaud's, in EM secondary to Raynaud's just chilly feet one day usually means burning feet in a close future. (the same day, the other, or even the next week.)

xoxo, Liz

Permalink Reply by clarissajo on February 6, 2013 at 8:10am
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Liz, I think you're absolutely correct in your interpretation of the article. And your English is superb! :)

That's very interesting that treating your Raynaud's has helped your EM.

I rarely have Raynaud's flares anymore, it's almost all EM now. But if my feet do turn cold and blue, I don't try to warm them as much as I might have in the past, because it triggers an EM flare down the road, as you say. And I never use extreme methods of cooling (such as ice), rather just leave feet uncovered as much as possible. I have noticed that ice or even cold water triggers worse EM later.

Thanks for all the info!

xoxo

JoAnn



liz said:

So first to JoAnn, ppl never pay attention about anything until they dont have to deal with it on "their own skin". So, I'm not surprised about what you're saying of ppl on forums. :)

Now to my post. From my understanding, the article is saying that superficially EM and Raynaud's may appear like completely different (oposite) conditions. One caused by vasodilatation and other by vasoconstriction. BUT that in cases when ppl having BOTH conditions, they don't stand like two different things, but are related. Means the worse your Raynaud's is, the worse will your EM get, simply like if the body is trying to "fix" the extreme cold by making it hot, but it kinda overreacts. That in these cases when patiens dealing with both conditions (EM is the more annoying one, no doubt about that) it helps (in my case it definitely does help) to treat the Raynaud's as a primary condition by using vasodilatators and that it simultaneously improve the EM. As they wrote "A mild vasodilator (to
prevent the original Raynaud’s phenomenon) can help some patients." (patients with both conditions)

It sounds very logical to me, tbh I always felt it this way, so for me the words I read were just reassuring information that I understand by body well.

Simply, if you have Raynaud's, Erythromelalgia can be just secondary to it. If you treat Raynaud's your EM goes away.

That's why my sympatectomy surgery (which dilates the small vessels) has such apossitive effect, despite I was more bothered by EM than Raynaud's before. "In erythromelalgia associated with Raynaud’s, the sufferer
does not necessarily notice the blanching of the digit or
limb but is merely aware of the hot burning extremity."

Using vasodilatators is definitely helpful in these cases. Let's call it EM secondary to Raynaud's.

I hope I wrote this at least a lil bit understandable, I'm not native English speaker, so sorry for this. :)

This all also means when you have EM secondary to Raynauds that every cooling down your extremities during the EM flare makes it all only worse, putting you into thevicious circle. The more cold the more hot follows. :)

For me this works on 100%. And dont forget it doesnt have to be just getting really cold and feeling really bothered by Raynaud's, in EM secondary to Raynaud's just chilly feet one day usually means burning feet in a close future. (the same day, the other, or even the next week.)

xoxo, Liz

Permalink Reply by liz on February 6, 2013 at 12:19pm
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thanks everyone for reactions :)

btw i just got home from a walk with my dogs, its horribly freezing there, so my hands and fingers were all red and frozen and painful and now as rewarming they on fire... oh wanted to say, never rewarm your extremities too fast or by using too warm water, it causes chilblains. the best thing is to squeeze em

Permalink Reply by NiceGuyDanny on February 8, 2013 at 4:27am
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Thanks for the info liz.

My rheumatologist totally ignored me regarding EM until I found this group! She would berate me for putting ice packs on my feet, not acknowledging--until now--that my hot symptoms weren't raynaud's. I've been generalizing the whole thing as Raynaud's for years because she has. I have severe Raynaud's, and I'd say severe EM. I've got a doctor's appointment this week. I'm just pissed--even when I was first symptomatic w/ Raynaud's, it took 3 winters before Kaiser even sent me to a rheumatologist. My feet are so bad, you guys. Just alien toes, so red, purple, pain. Time to get them up. And with the Raynaud's, the cold, I can control that so much better. But the EM, it's so tough because even just my feet being too warm in the winter because of layers, etc., they swell up so much, the heat is worse than the cold for me.

I find this interesting.

I think in some Raynaud's can make the EM flare but in others it is one condition that causes them both to happen independently. My Doctor calls mine a neurovascular instability. This isn't a condition or disease it is just a name for the combination of symptoms.
I think what differentiates one from another can possibly be found in what season is worse for EM. For some winters are the worst possibly because EM is a reaction to the Raynaud's encountered in winter. For some like myself summers are the worst. Any kind of heat over 65 degrees is a nightmare for me. Yes I still get Raynaud's in the winter but it is the lesser of evils and doesn't necessarily send me into an EM flare where for some Raynaud's is a precursor to an EM flare.

I can still get Raynaud's in the summer in the AC or when touching something from the refrigerator or freezer so it is a year round tight rope walk trying to find that 1 or 2 degree place where one or the other isn't triggered. If that isn't hard enough that small window of acceptable temperature changes from day to day and month to month!!!!!

Take care,

Alina
Nel said:

Clearly there are a lot of us who have both Raynauds and EM. Are you thinking that the EM is secondary to the Raynauds, or that one is caused by the other and which way round?
1 Like

My neurologist also calls mine a neurovascular instability.

It’s all very puzzling. In the heat of Summer I flare in temperatures over about 70 and in sunshine of course and with the use of fans etc. I can cope indoors in an English summer. Outside is a different story and to go out on a sunny day results in severe burning even in flipflops and only a hospital appointment will get me to leave the house. In Summer I stay in bed all night 3 nights out of 4.



It is difficult to explain why the Winter is so much worse. Central Heating makes me feel ill and itchy and brings up a rash so I have to keep the house cool. I can’t wear extra clothes because they will cause a flare and also bring up an itchy rash and huge heat in my legs, arms, back etc. But while my skin feels burning to the touch my body feels so cold that I shiver uncontrollably and ache from trying not to shake with the cold. in Winter I feel permanently freezing cold at the same time as I am trying to cool down my burning limbs. Obviously keeping the house cool triggers Raynauds attacks too, usually in my hands and chilblains on my toes. At night when I can’t sleep for the flares in a cold house with no bedcovers over me, I am to be found on the kitchen tiles with blazing hot feet and legs, wearing fingerless gloves on my dead white hands, and not a lot else. Fingerless gloves allow my hands to warm up gradually; ordinary gloves will deal with the Raynauds and then trigger an immediate flare. If I put on a dressing gown or anything more than a light tee-shirt the flare will keep me from my bed for the entire night, night after night.



To sum it up my core in Summer is comfortably warm and of course I don’t need layers of clothes. Then it is up to me to keep myself cool as possible, blocking out the sun and staying indoors. I do my gardening in the evening until the dark drives me in.



In Winter I have no control at all, freezing and burning all at once and feeling permanently ill from lack of sleep.



I hope that makes some kind of sense.

Me too, Alina. I've learned how to control the Raynaud's pretty good, but the EM is impossible here.

Alina Delp said:

I find this interesting.

I think in some Raynaud's can make the EM flare but in others it is one condition that causes them both to happen independently. My Doctor calls mine a neurovascular instability. This isn't a condition or disease it is just a name for the combination of symptoms.
I think what differentiates one from another can possibly be found in what season is worse for EM. For some winters are the worst possibly because EM is a reaction to the Raynaud's encountered in winter. For some like myself summers are the worst. Any kind of heat over 65 degrees is a nightmare for me. Yes I still get Raynaud's in the winter but it is the lesser of evils and doesn't necessarily send me into an EM flare where for some Raynaud's is a precursor to an EM flare.

I can still get Raynaud's in the summer in the AC or when touching something from the refrigerator or freezer so it is a year round tight rope walk trying to find that 1 or 2 degree place where one or the other isn't triggered. If that isn't hard enough that small window of acceptable temperature changes from day to day and month to month!!!!!

Take care,

Alina
Nel said:

Clearly there are a lot of us who have both Raynauds and EM. Are you thinking that the EM is secondary to the Raynauds, or that one is caused by the other and which way round?

Yes, clear. You're an awesome writer. And totally tapped into your system. Give me a lot to consider. Yeah, my legs streak with red in the heat too. My core is always warm. Totally confusing. Unless my feet are elevated, they are such scary colors, I can't even imagine wearing flip flops in public. I'm a confident person, but they look like I'm about to die, like something is so scary wrong. I don't know. My body will be in pain, though, if I don't move a lot...

Nel said:

My neurologist also calls mine a neurovascular instability.
It's all very puzzling. In the heat of Summer I flare in temperatures over about 70 and in sunshine of course and with the use of fans etc. I can cope indoors in an English summer. Outside is a different story and to go out on a sunny day results in severe burning even in flipflops and only a hospital appointment will get me to leave the house. In Summer I stay in bed all night 3 nights out of 4.

It is difficult to explain why the Winter is so much worse. Central Heating makes me feel ill and itchy and brings up a rash so I have to keep the house cool. I can't wear extra clothes because they will cause a flare and also bring up an itchy rash and huge heat in my legs, arms, back etc. But while my skin feels burning to the touch my body feels so cold that I shiver uncontrollably and ache from trying not to shake with the cold. in Winter I feel permanently freezing cold at the same time as I am trying to cool down my burning limbs. Obviously keeping the house cool triggers Raynauds attacks too, usually in my hands and chilblains on my toes. At night when I can't sleep for the flares in a cold house with no bedcovers over me, I am to be found on the kitchen tiles with blazing hot feet and legs, wearing fingerless gloves on my dead white hands, and not a lot else. Fingerless gloves allow my hands to warm up gradually; ordinary gloves will deal with the Raynauds and then trigger an immediate flare. If I put on a dressing gown or anything more than a light tee-shirt the flare will keep me from my bed for the entire night, night after night.

To sum it up my core in Summer is comfortably warm and of course I don't need layers of clothes. Then it is up to me to keep myself cool as possible, blocking out the sun and staying indoors. I do my gardening in the evening until the dark drives me in.

In Winter I have no control at all, freezing and burning all at once and feeling permanently ill from lack of sleep.

I hope that makes some kind of sense.




"Nel said:


In Winter I have no control at all, freezing and burning all at once and feeling permanently ill from lack of sleep."
Hi Nel,
That is how I've come to feel this winter. Winter used to be better than summer for me but something has changed this aussie winter. I still deal with 'sandpapered skin sensations etc. But what you wrote pretty much sums how I feel a lot of the time. I am permanently exhausted from not sleeping properly.
Please excuse any spelling mistakes. It's auto correct.
Blue

I have both, too. I have had Raynauds for about 10 years and quickly learned to avoid the cold. I gave up traveling to cold places, am have had to cover my hands when grabbing things out of the fridge, and always had an extra jacket/sweater just in case the air conditioning was too cold in a store or restaurant. For the past two years I have now been dealing with erythromelagia. My first bout came from what seemed to be lead poisoning. It seemed to go away when the lead levels retreated back to the normal range. Unfortunately, since January the erythromelagia has returned stronger than ever. I live in Las Vegas, so just walking in a parking lot is a challenge. I tell my husband (who is deserves a medal for trying to help me) that "I am a delicate flower!" I must be kept at the right temperature.

I am positive that the two must me connected in some distorted neuro-vascular dysfunction that has become my reality. Now if only I could find out why? Hmmmmmmmmmmmmmm.... :)