New EM last 6 months - likely post COVID

General
1

Hello everyone,

I am a mid 40’s male - I am new to the group. I am a scientist, I study how disease occur, so I have some insight into this disease of mine (ours).

I have new onset, relatively classic EM mixed with pernio/Chilblains. Essentially had no medical problems prior to this - played hockey, hiking, mountain biking, great shape, etc…

Earlier this year I had some very mild symptoms (headache, small fever) and had a borderline positive COVID-19 test when COVID-19 testing was just beginning (so accuracy of this is unclear). A few weeks later, I developed what looked exactly like COVID-19 toes. No EM symptoms, but toes were cool. A few week after that I noticed in retrospect the gradual EM symptoms starting, with intense heating of the toes.

It it is all in the toes (perhaps a bit of ear flushing). The toes are worse with heat, shoes, feet hanging down, worse in the am and late PM, and improved with raising feet (over head). I have symptoms evert day, multiple times per day. I saw everyone, as I am in the medical profession - every specialty, including remote visits with the folks at Mayo, other leading centres in canada and the US. All blood work, autoimmune panels, negative. I had a work-up for other causes of EM, also negative as far as you can tell. I had research blood work, that demonstrated some blood proteins are higher than they should be - but none of us know what to make of it. I wonder if others with EM / pernio have similar, but would need research study to test this.

I have tested mutation negative for EM, as I had some hot hands as a kid I recall.

Nerve conductions tests normal, however still waiting on results of small fiber nerve conduction tests (testing to heat/cold response).

I have what is now stable inflammation in 3 toes on the outside of each foot (symmetrical)…the two middle toes are spared from inflammation. The big toes are most sensitive to heat, become hot first, and without prompt raising, all the toes will flare with heat. The veins will get large in the whole foot if the feet are down, and the feet and even calves will hurt due the to rapid increase pressure from blood flow. I wear sandals only, and at home, no socks. If the feet are hot, and veins get engorged, the first 5-10 steps, there is intense pain, but as I keep walking (and walking causes blood to return from the feet to the hurt), the pain improves…so I need to keep walking and not stop. The toes are really hot still - but blood only leaves the feet if keep walking, or I stop and raise my feet very high (way over my head).

I have very small temperature sensors on my toes at all times (measure every 5 min) - and have seen that the temperature will rise 10 C (18 F) in 5 minutes - really rapid when a flare happens. It is really remarkable how fast it occurs. I cannot say having the temperature sensors has given me any insights into patterns though. My lateral toes can get quite cold, so it is like others (both too cold and too hots at times).

I tried steroids creams, other creams (ketamine/lido/amytriptaline), beta blocker creams, and oral steroids (6 weeks)- with no clear benefit. I do not have only EM, but also inflammation - that was the reason for the steroids. I had a biopsy of one toe - lots of inflammation, and was slow to heal from it. Steroids were started after this. I cannot help but think if steroids were started when this all began (very early, before the EM symptoms), I could have avoided these problems and the nerve damage…but that is a guess.

Other medications I have tried or am on - unclear if any really helped

  1. Propranolol (perhaps some very tiny benefit)
  2. Gabapentin at night only - during day no effect. probably no help at all.
  3. Vitamin D - no clear benefit
  4. Methotrexate (for inflammation) - unclear if working for that
  5. Zyrtec (old medication)…when I have changed to the dose, to change in symptoms.
  6. Recently misoprostol (400 twice daily) -like the old trial that showed benefit. No obvious benefit in terms of how easily toes flush, on week 3 of it.
  7. Sertraline…old medication --> changed to cymbalta. This happened while I had EM a few months ago. It could have correlated to slight worsening - although there is week-to-week fluctuations, so its hard to tell
  • for example, I have a recumbent bike I use to exercise
  • I used to go 30 min twice daily, get a good sweat each time. The temperature in the toes actually goes down with exercise like this. I did not get warm toes with exercise for a while. A couple of times, I noticed if the temperature started to rise in the big toe, if I jumped on the bike, it could reduce the temperature.
  • but after the change in this medication, I noticed my toes could flush with exercise
  • a challenge here, as I was coming off the oral steroids around the same time.
  • this taught me never to change two medication near each other as I a left wondering if it steroids did help in this small way, or it was sertraline, or it was random.
  1. Magnesium - for some reason, felt terrible (not the toes), but whole body wise after I tried this a few times (every time). Perhaps a different prep will be better.
  2. Oral nifedipine - no benefit. In the very beginning - there was more cold toes than hot toes …so we tried to dilate the blood vessels…

I spend most of the day with my feet up (above the heart level), and work from home at a desk. Alas - a massive change in life style, quality of life for me and the family.

I feel like the inflammation from the viral infection lead to nerve injury in the toes (COVID toes), which then lead to EM. I have spoke to heads of dermatology departments that study COVID19, and its seems they have seen this temperature instability in those after COVID-19, so there is some cold/warm dysregulation in the toes - but usually, if not always goes away. In my case, it did not.

So - I am working with a rheumatologist/s to calm the inflammation in the toes - then deal with the EM afterwards. Since oral steroids did not work, I am not overly hopeful there. The next med I will add is hydroxychloroquine for the inflammation.

My EM+pernio, we call vascular instability. That is, it can become too hot or too cold, too much blood flow or not enough to different regions of the toes.

Medications i am thinking of trying.

  1. Mexiletine is on the horizon for me. I will do this after I exhaust efforts to reduce toe inflammation
  2. CBD - why not?
  3. There is a new class/es of medications called SGLT2 inhibitors (or GLP1 agonists) - and they work for some diseases (diabetes and non-diabetics) quite well. One potential target of these medications are blood vessels. I figure why not as well.
  4. When my inflammation better, I will try hot soaks (Bob’s protocol) with or without the capsaicin cream, but because of tender/inflammed skin, I cannot
  5. Light therapy, as previously posted here.

Of course, little of this is evidence based - but what can we do?

2 Likes
2

For any of you interested, I posted an image below of temperature readings over time from 2 toe (left and right big toe). Grey areas are sleeping…it is normal for temperature to increase with sleeping like this. I sleep with my feet on 5-7 pillows.

During the days you can see the temperature can go up.

Slide1
Slide11280×720 100 KB

If you are interested - temperature sensors are called ibutton thermochron. Easy to use and hook up to any computer through an adaptor - relatively cheap as well.

image
image864×236 80.6 KB

3

I’m a bit confused. You keep saying you have inflammation but that all bloodwork and autoimmune panels are negative. Inflammation is immune mediated erythema. Do you have markers for elevated immune involvement or are you just incorrectly using erythema and inflammation as synonyms? (They’re not.)

The erythema seen in erythromelalgia is not inflammation, which is why things like NSAIDs, steroids, and immunosuppressants (such as methotrexate) are ineffective treatments for erythromelalgia.

4

I had a biopsy of one toe - dense inflammatory cell presence all through the various layers of the skin and beneath. So there is an inflammatory component in some of the toes, but not in others others.

Its not classically autoimmune - all tests are all negative - there are no more left to run.

Standard inflammatory tests (CRP, ESR) are normal, but the biopsy speaks for it’s self - it was not subtle.

Research panel of 80+ circulating cytokines/growth factors, the inflammatory cytokines were normal - but the growth factors were elevated, related to whatever “localized inflammatory” process I assume is happening in my toes. My next step is to sample blood draining from the toes and compare to the something from the arm, to see if the elevated growth factors are coming from there.

So i get the classic erythema associated with EM with the flares starting with big toe–>small toe, that as you point out is not inflammation at all.

However, in the absence (or in between) flares, I have residual, constant erythema the lateral toes of both feet. Thus, its both, underlying constant, grumbling inflammation in the lateral toes, and classically triggered EM in all of them that comes and goes.

So the methotrexate is just for the constant component…not the flaring.

Superimposed

5

I’m not sure I really follow your thought process and don’t know if the biopsy could have been Incorrectly interpreted. Erythromelalgia is a very rare disease. When I initially began with symptoms I had an MRI. The radiologist interpreted the results as synovitis (inflammation of the synovial capsule). That was incorrect and I chalk it up to the likelihood the radiologist had never seen an MRI from an EM patient before.

I was put on methotrexate initially, along with a corticosteroid, and I was never worse than when I was taking those drugs. They don’t work for EM.

6

HI Steve, sorry you are joining our club. Loved that incredibly detailed description. EM is nothing if not interesting.

My onset was after breaking a toe (spiral fracture) and before that I had been in good shape too and it is also idiopathic. My doctor says that it was likely I was headed this way anyway and the fracture just pushed me over the cliff. Now I have it in my hands and feet. It took a few months to stabilized. Recently (I am in year 5) I have developed some Reynauds and have the surreal experience of flaring and feeling cold at the same time.

I am on lyrica for the inflammation, and sidenafil and aspirin for blood flow. Lyrica has been a life saver. I tried the generic but apparently different formulations matter for our disease. FWIW, steroids make me worse.

It seems like everyone has some tricks that have helped them. A few things I have found to be be very helpful include:

  • Exercise helps me even if I do flare. But for the most part, as long as I avoid friction on my feet, I won’t flare while exercising. Sometimes I flare afterwards but it doesn’t really hurt that much, which is weird. So biking, swimming, rowing are all great for me and seem to help over all.

  • I have also found that stopping eating about 5-6 hours before I go to bed helps with night flares. It also allows me to stay on my feet longer in the morning (making breakfast, getting kids ready for school) before I flare.

  • Paying close attention to my diet - some foods trigger flares.

  • this is going to sound stupid but laying down and propping my feet up on the wall so that I am in a pike position.

  • and finally, when I flare, a bucket of room temperature water helps enormously.

7

been thinking about this a bit more - I am not sure that treatments for standard inflammation work for EM. Maybe they do but that has not been the approach of my doctor who is working on nerve overactivity and blood flow.

Also, very interested in the relationship between Covid and EM. My understanding is that a trauma can bring it on. Was it that aspect of covid or something particular about the virus that triggered the EM (in your thinking).

8

Thanks Jalika,

I do the pike position quite a bit actually!

I have not been able to figure out my triggers in the food - although I will try to keep a better track.

Can I ask what you mean by inflammation being improved with Lyrica? What aspect did it help with - pain, the temperature changes? Gabapentin (300 mg three times daily), did not help with temperature flares, etc. Since the temperature naturally rises in the toes at night (for all people), it could also causes a flare, and cause pain the toes that look inflammed - so gabapentin helps a bit with the pain at night for me.

I’ve included pictures below…the 3 toes laterally are constantly inflammed. They are not warm - they are often cool, with skin breakdown, at times very small wounds. The two medial toes (no inflammation), but the big toe gets warm. In the pictures here, I had an ingrown toe nail that made things worse for a time.

The shoes are hard to wear in great part, because of the lateral 3 toes - they are tender. The other two- which look normal, skin is normal, are not tender - are much more sensitive to temperature changes, and are the ones most affected by EM.

You can also see the temperature probes here.

I think with COVID-19…look up COVID toes. That is what i had in the lateral toes. They were simply cold (or normal temperature) in the beginning - I think that was form of trauma to the toes. I did also “smash” those toes playing hockey around that same time - I wonder if they were vulnerable and that exacerbated the process in some toes.

Carter- the steroids, MTX - no benefit on the EM in the medial 2 toes. I am hoping they will help with lateral 3 toes that this skin level scabbing, inflammation.

For

Left foot Time line
Left foot Time line1280×720 161 KB

9

Just out of curiosity, have you had ingrown toenails in the past or did that start post COVID toes/EM?

Years ago I started having ingrown toenail issues with my right big toe and I’m not sure if the ingrown (or subsequent surgery) caused EM or if EM started first, which lead to swelling and the nail breaching the skin.

Site member “MNP” documented his ingrown toenail surgery and EM, here is his blog: http://erythromelalgia-in-toes.blogspot.com/2015/

10

By the looks of your photos and description of the redness not being warm but rather cool, my best guess would be that this is not EM. This looks like chillblains which could be secondary due to post-viral inflammation from COVID. This would also cause hyperemia when exposed to heat which you could be confusing for EM. Your toes likely have poor circulation from the inflammatory damage to small fiber unmyelinated nerves, which has likely subsided since your ESR was normal but your small fiber nerves might take months or longer to heal and recover proper perfusion control. Even a punch biopsy would have a hard time picking this up but you say it did so I am pretty sure that is what this is. Hope this helps a little.

1 Like
11

I was interested in this post bc I have tried everything listed but CBD & lyrica… going to have my doc get more info. I’m really just looking for pain relief. It’s unbearable and has gotten worse over the past yr. (symptoms started 3yrs ago) one out of 22 doctors figured out it was erythromelalgia. now it’s went to my pinky and ring fingers r 50% less feeling than other fingers. I Didn’t feel them poking the needle all the way to my knees. I almost killed myself this year bc my body was over being in shock from pain all the time. I don’t believe in it but the majority of days my flare up I wish I could goto sleep the whole time. I was excited for the QSART test and biopsy of my feet and legs on the 28th but looks like I’ll have negative results? I haven’t tried going against the wall then putting my legs along the way. Going to have to try. My flare ups were 4months past 2 yrs then this yr it has lasted almost 10months so far. Any advice? What helps with the burning and pain of feet? Does it move to your hands fast?

12

Thanks Joe, good points. I hope its like the nerve injury that just takes a long time to heal…rather than some permanent situation.

I agree - the lateral toes are certainly Chilblain/Pernio like. I would also say that many diseases are a spectrum of disorders that look similar to each other in terms of symptoms, but the underlying biological causes can be different.

For me, I would say my medial toes, that lack any baseline inflammation, redness - are an area where redness/erythema / swelling / heat - come on together and are only present when triggered in your typical EM fashion (friction, pressure, heat, having feet down, etc). The medial toes are behaving very differently than the lateral toes.

Interestingly, the medial 2 toes and the lateral three 3 toes have two different nerves that supply them. My own speculation is that nerves of the medial toes were symmetrically affected in the absence of any obvious inflammation, leading to classic EM symptoms - in the absence of an EM mutation. The lateral three toes are inflamed, typically cool, but can warm up if the medial toes warm up first…so there is mixed temperature instability and chronic inflammatory damage in toes.

The inflammation is very interesting when in happens…even in individual toes - it can affect exactly the left or the right half - following nerve/vessel distributions.

Dr Davis - from Mayo, the guy that little wrote the book on this, told me he has numerous patients, who in the exam room, have cold toes on the left and hots toes on the right - and that during visit, the temperatures would reverse - so very dynamic process.

CharLilLnn - I am really sorry to hear about the severity of your symptoms - from the information you gave, I cannot tell regarding whether you have EM or not.

Are your flares 24hrs per day when you have them, is it worse with heat, keeping the down, and improved by raising them?

For my toes (big toe on each side), when I sense either are getting warm, I quickly put my feet in a way that gets them fairly high (lying in bed with 7 pillows, or a good reclining office chair with pillows stacked on my desk)…it typically works quickly for me, and temp improves. I have the luxury of working from home these days, but if I didn’t, it would be miserable. It has not spread to my hands obviously, but I do have some hand involvement that is typically minimal, from time to time. I imagine any variation is possible with fingers and toes.

If you are having neuropathic pain (tingling, sharp pain, electrical pain), there are large variety of medications that can be tried. Certainly lyrica can be tried instead of gabapentin - but others as well. Some people find improvement with that switch.

For me, the burning comes directly form increased blood flow, and continuous heat that the blood generates over a period of time…the toes feel scalded and I feel the neuropathic pain only then. So for me, its to prevent the rapid heating, and minimize the time of the toes are hot , and all I have for that is raising my feet… I do not experience neuropathic pain except in those circumstances. Hang in there!
s

13

Kost - the ingrown toe nail was my first in my life, and about 6 months into journey with this condition. Never had any toe/feet problems before. I had come off high dose steroids about 1 earlier, and was on methotrexate as well - so perhaps immune system suppression also played a role.

The foot / wound care team is really aggressive at my hospital - eve in the uninvolved toe, they have done a lot of work to try to prevent more episodes. They did a lot of filling, etc…and also put small thin bands of cotton along sides, and underneath the toe nails on both sides (essentially a buffer between skin and toe nail. Every time I go and they work on my feet, sets of a very uncomfortable, hrs long flare - hopefully this will prevent future trouble.

For me - the flares - I can live through them with respect to the toes that lack inflammation (2 medial ones). They are health toes. But the flares damage my lateral toes each time they happen, making them worse - that is my real problem at the moment…

14

I tried many things of the same treatments for 3-4 years. The only one that helped was and is cyproheptadine twice daily. I then discovered that magnesium had some positive effects (mg plus protein my Miller phamacal) from Amazon. In addition to 650mg aspirin a day. The aspirin leads to definite improvement. If I reduce dosage of miss a few days the flares worsen again.
I hope you find great relief.

15

Thanks herdfan.

The cyproheptadine is one I am also thinking of as well. Its complicated since I take an SNRI (duloxetine) - which increases serotonin levels and cyproheptadine will block it…it is said that some people respond to increased serotonin levels, while others respond to decreased levels…

I have not tried aspirin at such high doses. Do you have increased platelet / red blood cell counts in the blood, as those with increased counts are the ones that typically respond to aspirin?

Thanks for the Mg suggestion - should I try one that may be better tolerated by me.

s

16

Yes 23:55hrs a day it’s constant burning w/ tingling, prickers, and randomly usually one every 30 secs is this stabbing sensation and I scream out in pain. It’s hot to the touch but then b4 or after flare they become freezing cold n I can’t get warm, I was sleeping outside for months on my deck cuz the cold feels better. But now I guess I messed up my kidneys because of cold exposure. My friends even bought me a pair of cold neuropathy socks that have ice packs on top, bottom and back of ankle. They said I have the EM I had to go round n round w/ 26 doctors until my primary doc figured out what I had & was familiar w the disease because she ran into a case a yr ago. They have tried tons and ton of muscle relaxers, nerve pills, patches, pain pills, Lotion, other meds. Gonna look into the C-word & ask about Lyrica. I also saw on here ketamine infusion work, lidocaine infusion, CBD I need to try. I’ll try ANYTHING. Does it get better or way worse. It’s gotten more than double than the previous 2 years. It went from 4months flare then bare able pain now this yr it’s goin on 11 months. I feel like I shouldn’t exists. Like god is punished me for now reason this even more. Hope ur ok at the moment & stay strong we can beat this horrible disease.:kiss:

17

Hi Charliynn

I can see its a very difficult situation. If the symptoms are localized to your legs, there is also a nerve block option that could be tried for EM - although as far as I know its for primary EM. I’ve included a link to a case report below. Perhaps discuss this with your doctors to see if it could help for your case.

https://www.jaad.org/article/S0190-9622(13)00781-0/abstract
" Erythromelalgia is a rare clinical syndrome characterized by intermittent redness, increased temperature, and pain, usually of the extremities. It may be associated with postganglionic sympathetic dysfunction. Treatment can be challenging and refractory to conventional modalities. We describe a case of primary erythromelalgia refractory to medical treatment that successfully responded to sympathetic nerve block using bupivacaine."

18

My doctor keeps telling me that lyrica basically calms the nerves so that they stop doing their idiot dance with my capillaries to trigger the flares. (the last bit was me and I should probably ask about it more specifically). What I have found is that lyrica doesn’t stop the flares. It simply makes them hurt less, remarkably so. Situations that used to be unbearable (i.e., going to my kids’ swim lessons after teaching a class in the late afternoon) became manageable. That particularly situation was unbearable because any sort of walking causes friction (flares), stress (teaching a class while fun is hard), and the humidity at the pool.

19

It’s traveled to 2 of my fingers on each hand. Ring and pinky. My hands will be cold or my fingers tingling. Haven’t tried to warm them up I just use “frozen” socks when my feet are on fire. Thx for some advice. I hope you get the help you deserve like the rest of use. I’m just sick of feeling helpless n hopeless bc all the doctors are making me feel crazy. The one that figured out I had EM hasn’t even tried to help. She yells at me for wanting her to help me with pain, bc of people that abuse them but I NEED something, anything that will help with the pain even if a little

20

Hi Steve, I am sorry you are experiencing this. It’s downright awful to have your life interrupted in such a painful way. I am a nurse practitioner with a diagnosis of EM. Post COVID temperature dysregulation is interesting. I wish we understood more about it. When my EM was at its worse due to inflammation due to another disease process, the things that helped the most were not prescription meds suprisingly. I saw a functional medicine provider who had me stop eating gluten and dairy. He also started me on Gaia Curcuma NF-kB liquid phyto caps (its a curcumin supplement) 2 tabs 2x daily and high dose fish oil- OmegaGenics EPA-DHA 720 2 softgels 2x daily (anti-inflammatory). I too had been to the mecca of Mayo, had the ketamine cream, and left disappointed. Within 48 hours of changing my diet and start of supplements, my flares went from constant to intermittent and have continued to improve. I am assuming this occurred due to lessened inflammation in my body. I did do heat desensitization but had to get the inflammation way down before that was a possibility. I’d say I am 95% improved and only deal with a brief 10 minute flare maybe once a month now. As a medical provider, I have to say I tried the diet changes and supplements because I was super desperate. I wasn’t convinced it would help, but I stuck to it. It was a game changer for me.
Yours in hope,
Lauren Sparks

1 Like