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
- Propranolol (perhaps some very tiny benefit)
- Gabapentin at night only - during day no effect. probably no help at all.
- Vitamin D - no clear benefit
- Methotrexate (for inflammation) - unclear if working for that
- Zyrtec (old medication)…when I have changed to the dose, to change in symptoms.
- 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.
- 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.
- 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.
- 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.
- Mexiletine is on the horizon for me. I will do this after I exhaust efforts to reduce toe inflammation
- CBD - why not?
- 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.
- 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
- Light therapy, as previously posted here.
Of course, little of this is evidence based - but what can we do?