This is a lecture on SFPN by Dr. Oaklander of Massachusetts General Hospital. Dr. Oaklander states that 50% of EM sufferers have SFPN. It is a very interesting watch. Dr. Oaklander is speaking at our state awareness day.
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If you’re attending the awareness day, my question for Dr. Oaklander would be —do EM patients have EM because they first had SFPN or do EM patients develop SFPN as a consequence of EM? If SFPN was the causative trigger, wouldn’t we see a lot more cases of EM?
That is a great question and I do believe that EM comes after but I’m not positive and I am collecting some questions that my team on the audience will ask. This will be one of them. It will be video tapes to so others can watch after. Thanks for your question!
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Hi I have CIPD - Chronic Inflammatory Demyelinating Polyneuropathy which is being treated with IVIG treatments weekly. I developed erythromelagia as a secondary disease to the CIPD.
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That’s great! Thank you for passing my question along!
found the slides from her lecture, just in case it interests people. Found them super helpful and interesting 
This was wonderful to share. Thank you!
Just to let you know…small fiber neuropathy would be the cause of the EM so if small fiber is treated successfully then EM should be better or in remission. So small fiber comes first but it does’t always test positive right away.
Interesting. Then treatment for SFPN could be a potential future treatment option for EM. Also, since SFPN is much more common than EM, it’s more likely to be researched. That should provide hope to those struggling with idiopathic EM today.
Thank you for posting this. Very interesting!
It actually is being researched by Dr Oaklander and the team at Massachusetts General Hospital. I do believe all EM is secondary to a cause so EM in this case is caused by SFPN. EM is really just a symptom or a condition and not a disease even though it is listed as a rare disease.
I think for most people erythromelalgia is like anemia, a symptom and not a disease. But hasn’t primary erythromelalgia been proven a disease? It has an identified cause, mutation in the SCN9A gene. Just because it’s monogenic doesn’t make it not a disease. Sickle cell disease, cystic fibrosis, and Tay-Sachs are all monogenic diseases.
To clarify medical terms: a “symptom” is a person or patient’s complaint eg I have pain, a sore knee, I feel nausea, I’m anxious.
A “sign” is a finding on examination eg red feet, Temperature of 38C (for those who use centigrade), BP 120/80 etc
A “condition” is the lay term for a “diagnosis” eg
Primary erythromelalgia or
Secondary erythromelalgia due to whatever eg a rheumatic or haematological disease
A “differential diagnosis” is a list of different diagnostic possibilities which need to be considered before firming up THE diagnosis.
eg chest pain could be due to heart, lung, gastrointestinal, musculoskeletal or psychological causes.
A diagnosis of Small fibre neuropathy or SFPN may be primary (possibly autoimmune or dysimmune), idiopathic (we don’t know, “yet!”) or secondary due to some other disease process.
The seemingly common occurrence of SFPN in EM is a tricky one… what came first, the chicken or the egg? Does the underlying pathophysiology (malfunction) causing EM also cause SFPN? Clearly there is a close association.
Tricky. At least we have smart neuro scientists like Dr O and others who are onto it. Most of this stuff was unknown back when I studied medicine, indeed it’s leading edge research and a great example of how one learns more after graduating than one ever did before.
EM by itself needs to be further sorted out into primary or secondary.
Dr. Oaklanders’s position is that EM is secondary period. She encourages all to find the cause and push your doctor for it. However, it is easier said then done.
I had top doctors say it is more of symptom than a disease. They all have their opinions and much more research is done for us to have the absolute correct information.
But at any rate we have it and want a cure !
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I found this presentation to be a saving grace for me as I showed it to my doctors and it helped my doctors give in and test for SFPN and the SCN9A mutation. Coming from a medical and neurology background, I find it extremely disturbing that the first 6 or 7 doctors I went to had never even heard of EM. My SFPN test was positive in my left foot, where my EM started, and was negative in my right foot, where I also have symptoms. I think this shows that EM can be a result of SFPN but also that is does not always have to be where to symptoms are located as my SFPN is only in my left foot but I have symptoms bilaterally in my hands, knees, feet, and ears. My understanding is that EM is a symptoms of SFPN and there is always a cause for degenerative nerves and contrary to many articles about how SFPN can’t be cured, if it is inflammatory or caused by a genetic mutation, treatment can actually cure the neuropathy and/or put it into remission to allow the nerves to slowly regenerate which can take years. Best wishes to everybody searching for their answer, I feel like I am one step closer to mine and it has put my mind at ease.
Well, thanks to my wonderful neurologist who fought for me, my insurance actually approved IVIG (what Oaklander advocates in case there is inflammatory origin) on appeal for painful idiopathic small fiber neuropathy.
I can’t find much on the forum about it. If anyone has any experience with it, I’d love to know. I received my second iv today and tolerate it fine. I have no idea how soon to expect improvement or what though (if it’s gonna happen).
My neurologist has me at a low dose (50) per iv, but my nurse said some of her patients are on 600 (no idea the units). She says she hasn’t seen anyone receiving IVIG be able to stop it (that it’s more like an indefinite thing).
Anyway. Glad to be trying. Take care everyone.
edit - Since I had arranged an at-home infusion of IG monitored by a nurse, I wondered if it’s possible at all to do something similar with lidocaine. Anyone know? I’m still fighting my insurance for an in-patient 5 day lido infusion. My derm thinks that both IVIG and lidocaine would be worthwhile, they might each be able to offer something the other cannot. Trying to schedule regular outpatient (in-clinic) lidocaine iv’s in the meantime but long wait list at my pain mgmt center for this.
Hi Joe, wondering what your SCN9A result was?
I think SFPN is very closely related to EM & may well be the trigger when the neurodegeneration crosses a threshold. What is causing the SFPN is the key question.
I have GAD abs & LADA. I suspect the GAD abs may well be causing the SFPN> symptomatic EM ie. its auto immune. Rather than the LADA, as my HBA1Cs have always been normal.
The auto immune basis may well be common to many of us with EM.
I’m currently exploring / trialling the anti inflammatory benefits of a WFPB diet. See nutritionfacts.org which is very compelling.
After just 4 weeks my previously weak pedal pulses (PT & DP) are now strong like they were many years ago. Given that a whole food plant based diet is the only diet with evidence of prevention and reversibility of CVD and the strong links between meat & diary & cancer it makes sense to try it also for its auto immune reduction benefits too.
Mine was negative and a second test for SFPN was normal for me. I have found relief in my EM with serotonin antagonists so my EM seems to be more vasospastic rather than a neuropathy.
To answer the earlier question regarding lidocaine infusion and IV I G. I do have the sodium channel Gene mutation and had a two-week remission while on IV I G. But then it failed me and we could never get it back in remission. Also tried the lidocaine infusion but after a few days it was apparent that it was not helping me. Just wanted to share my experience .