Another Diagnosis: Erythromelalgia

Like many of us, I already have an alphabet soup of diagnoses that seems to grow every year: ME/CFS kicked everything off almost 15 years ago. It causes Orthostatic Intolerance (OI), which for me includes both POTS and NMH. Plus I recently realized I still have Lyme disease which seems to flare up every few years.

Classic EM on toes & feet (mine is much milder)

Now, I can add one more to the list: Erythromelalgia or EM (yay, another acronym!) This started 3 years ago but was just diagnosed at the end of last winter. Because mine goes dormant in the warmer months, I have just begun to read and research EM, to learn more about it and how to treat it because it gets very painful at this time of year. EM is likely caused by/directly related to ME/CFS in my case, so it is possible that some of you may have it as well and quite likely if you do that it hasn't yet been diagnosed. It is considered a rare condition.

According to the Erythromelalgia Association:

"Erythromelalgia is a rare and frequently devastating disorder that typically affects the skin of the feet or hands, or both, and causes visible redness, intense heat and burning pain."

My EM began abruptly almost 3 years ago. It was the first week of December, the weather had turned cold, and my family made our annual trip to a local Christmas tree farm to cut down our tree. It was, as always, a pleasant time with my family, except that by the time I got home, my toes were burning with pain. I was desperate to tear off my warm socks and boots and was almost in tears. From that day on, the burning pain and redness in my toes continued until spring. The podiatrist guessed maybe it was a fungal infection, but the antifungal cream he prescribed did nothing. My primary care doctor had no idea what was wrong (is this sounding familiar?).

On my next visit to the dermatologist, I showed him my toes and described the pain. He said it sounded like chilblains. I told him someone else had suggested that, and I had looked it up, but my problem seemed to be the opposite. Apparently, with chilblains, cold temperatures make the condition flare and it feels better if you keep your feet warm. I had discovered, by trial and error, that heat made my feet worse. I explained to him that the only relief I got was from soaking my feet in cold water, which led him to conclude this was definitely not chilblains. He was also stumped, but - to his great credit - he didn't give up on me. He went off and did some research and called me back to his office.

He said he thought he'd figured out what I had: Erythromelalgia. He'd found information online on the Mayo Clinic's website, and he let me watch a video (at the link) about it on his tablet. He was right - it sounded exactly like what I had! Unfortunately, since it was a relatively rare condition, there didn't seem to be a lot of information on treatments. He prescribed a cream for me for the pain & burning feeling and advised me to use cold water soaks sparingly (and cool, not ice cold water) so as not to damage the skin. I limped (sometimes literally) through the rest of the cool season and breathed a sigh of relief when it disappeared again in the warmer months.

Two weeks ago, it began to flare up again - right on time as the outdoor temperature cooled. By the way, my seasonal fluctuations seem to be unusual; for most people, summer is worse, and the warm outdoor temperatures cause bad flare-ups of their EM. My theory, from what I've read and the photos I've seen, is that mine is milder, and it flares up when I start to wear shoes and socks in the cooler weather and switch from flip-flops to slippers indoors. This time when it started, I looked up erythromelalgia online and began to educate myself about the condition and its possible treatments.

For starters, my EM is almost certainly related to ME/CFS. ME/CFS causes autonomic nervous system dysfunction, which causes OI and can also lead to EM. Interestingly, I already take several medications for ME/CFS that are sometimes used for EM (beta blockers for OI/POTS and tricyclic antidepressants - low dose at bedtime - for correcting sleep dysfunction). Perhaps that is another reason why my EM seems milder than most and goes dormant for part of the year.

I was happy to find that there have been some studies on various treatments (though not many), and I eagerly read all that I could find on the subject and made a list of what might work for me. They divide EM into two categories: primary, which has a genetic marker, and secondary, which is caused by other conditions. I think with ME/CFS and its autonomic dysfunction, I fall squarely in the secondary camp (I also checked my genetic results and I don't have the marker). So, that somewhat limits my treatment options, but there are several things I'd like to try. My dermatologist doesn't feel qualified to treat EM - he'd never even heard of it before he dug it up for me! - but I have learned there are two potential doctors sort of nearby - one at Temple and one at Johns Hopkins - who might be able to treat it. So, that's my next step.

Besides digging up all the information I could find, I also joined two Facebook groups on erythromelalgia. I know from experience that nothing beats talking to other patients in the same situation about what has worked for them (that's where I found out about the two doctors in the area).

So, briefly, erythromelalgia symptoms often include:

• Burning pain and redness on extremities - often toes and feet, sometimes fingers and hands, and occasionally up the legs or arms, too. For some people, it also affects the face and ears.
• Some swelling and inflammation of affected extremities.
• Cold makes symptoms better; heat makes them worse - this is key and differentiates EM from many other possible diagnoses.

I miss my whimsical sock collection!

First-line of defense is simply keeping the affected extremities cool. For me, this means going barefoot or in flip-flops all the time in the house, keeping the house temperature cool (at least until my husband gets home in the evening!), sleeping with my bare feet outside of the covers, and occasionally - not too long or too often - using a brief cool water soak when the pain gets unbearable. It's not easy having cold feet all winter long, but it helps a lot! I am already missing my slippers and all my cute, colorful socks that I used to love wearing. Going out is tricky for me at the moment. Wearing loose slip-on canvas sneakers without socks seems to work best, if I really can't bear being out in flip-flops. Right now, my pinky toe is swollen, so even those hurt a lot.

If you think you, too, might have erythromelalgia (I'm getting better at saying & typing that!), here are some resources - information and groups - to help:

• National Organization for Rare Disorders (NORD) - information on erythromelalgia - a thorough summary of EM, plus an excellent list of related disorders that should be ruled out.
• The Erythromelalgia Association (TEA) - I was so excited to find out EM has an association! The website include basic info, links to more resources, membership info, plus a list of research studies.
• emedicine/Medscape overview on Erythromelalgia - an excellent and comprehensive explanation, with the best treatment summary I have seen, including a listing of all relevant past research that you can click through to for details. This is where I got most of the treatment ideas for myself.
• Mayo Clinic's Erythromelalgia Specialty Clinic - this is the page with the explanatory video that my doctor shared with me. This is the only EM clinic I have heard of so far. Just be aware that you should NOT seek help for ME/CFS at Mayo! They use the UK approach, which means only offering Graded Exercise Therapy and Cognitive Behavioral Therapy as "treatments" (which they are not and GET can actually be harmful). But, if I were in the area, I would definitely try the EM-specific clinic there.
• Facebook Group: Erythromelalgia - Medicines/Treatment, Information and Support
• Facebook Group: Erythromelalgia Support, Information and Awareness

So, I'm curious, does anyone else with ME/CFS or Lyme have EM or think they may have it? So far, in the two groups I joined, there seem to be quite a few EM patients who also have ME/CFS, POTS, or Lyme disease.

UPDATE February 2020:

Back in 2017, I successfully put my EM into remission using heat desensitization, a simple process of resetting your nervous system by gradually getting your affected parts (feet, hands, etc.) used to tolerating heat again. You can do this with hot water soaks, starting at about body temperature (98-99 F) and slowly and gradually working the temperature up (but no higher than 120 F or you can scald your skin). I explain more about my own process in Heat Desensitization - EM in Remission! and that post includes a link to the EM group where I learned about heat desensitization and many, many other people describe their own experiences. My own EM would flare up every winter; I have now been through my 5th winter with no EM, wearing warm socks, shoes and boots, and even sleeping with my feet under the covers!