Hi rie,

Absolutely.

Isn't this what we try to get through to patients every day ?

This is why I shudder when I think about all these "toe-nail cutting services" by Third Sector Providers

Dido

 

As someone who lives with a partner suffering from Erythromelalgia (EM), I feel a few comments coming on.

Sometimes EM is treated as a specific disease. This often ends up with patients being trialled on aspirin because the doctor has read some reports of that helping. But it looks like aspirin is only effective in those for whom EM is secondary to something like polycythaemia vera. In others it makes them worse. Similarly for propranolol (its effect on EM seems to be unrelated to its function as a beta blockers; no other beta blocker has any effect on EM).

But when treated as just a ragbag of symptoms that misses many of the common threads. The fact that there is a disproportionate mention of thyroid issues. That of the various medications available each seems only to help some - and to harm others. The relatively recent theories about Nav1.7 sodium channel issues.

But the cardinal feature of EM is heat. The skin is often objectively warm. (With a cheap infra-red thermometer we easily read 35 on the sole of partner's foot while raised on a footstool. Can't seem to get a reading above 32.5 anywhere else on the outside of the body. Higher temperatures have been reported in the literature.)

And pain. All patients report considerable pain. Partner and I agree that the pain might even be caused by the excessive heat.

The most obvious response of anyone with painful burning feet is to cool them down - often doing things like soaking in ice-cooled water. (And that gives rise to many other problems.) The second response is to avoid wearing shoes and socks even in winter. (In the Russian literature there are many descriptions of the old people who walk on the snow barefoot due to EM.)

And the most obvious response of feet exposed to such cooling is to develop chilblains/pernio. (Can't see any way to decide whether EM sufferers are intrinsically more prone to chilblains or if it is simply how they try to avoid over-heating.) Chilblains do appear to be a major topic among EM sufferers.

Those with a professional interest treading here may see a disproportionately low level of EM sufferers. The idea of anyone doing anything to partner's feet horrifies her.

EM can appear in feet, hands, face ears. One sufferer says that when she raises her feet (elevation to heart level often helps) the EM seems to occur in her groin. It is often progressive with the start being a touch in one foot and ending up affecting both legs to the knees or higher.

EM is highly episodic. Flares occur anything from minutes to days apart and of similarly varying duration. And the distress and pain does not disappear as the redness fades - the feet can be painful all the time.

The lack of obvious heat radiating from the feet in the case report immediately precludes EM as likely diagnosis.

What to do if you get a patient suffering from EM?

1 Refer to the local NHS dermatologists. (Partner was seen by the Bucks Dermo Review Clinic where around 15 doctors came and looked. And pronounced unanimously 'EM'. And passed her back to GP for 'care'.)

2 Tell the patient to join the EM and EMSupport groups on Yahoo. Introduce themselves and get posting. This is the most important single thing you can do.

3 Possibly mention The Erythromelalgia Association. For various reasons this is not very helpful but the patient should know that it exists.

5 Try elevating feet, e.g. on a foot-stool or on the arm of a sofa. The fullest relief occurs when the feet are at the same level as the heart - or very slightly higher.

4 Partner is on NO medication. The only thing she uses is CCS Foot Care Cream as and when the surface of her feet gets grainy and dry - likely a side effect of overheating. This does help with that symptom.

5 Tell them that as it has an incidence of 1:40,000 to 1:400,000 they are unlikely to find a single medic who really has experience. And even if they do, they are likely to be pigeon-holed into being the same as previous patients - even if they react very differently.

6 If you are within travelling distance of Dundee, consider a referral to Prof. JJF Belch - the *only* British medic I have identified with significant experience of EM.

Finally - EM often occurs in combination with Raynauds, chilblains, RSD/CRPS. And both CRPS and EM were both identfied by Silas Weir Mitchell.

Feel free to contact me if you wish. I am not a regular reader but do look in from time to time.