A 43-Year-Old Woman with Painful and Discolored Toes

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Pernio

 

I agree it sounds like pernio to me

 

How about Raynauds Phenomenon with further investigation for Scleroderma?

GB

 

All,
I'm with George on this one - Raynauds Syndrome.

Dido

 

Ditto Dido

 

Chilblains must surely be the most likely diagnosis? What are we told?" if you hear hooves think horses not zebras.


Cornmerchant

 

Hey CM,

Y`know the horses `n hooves are right...(cheers Ivan :drinks) The red herring was the fact that this pt was presenting in `the rheumatology clinic` which kinda infers an underlying systemic pathology. However, upon further reading of the Medscape article...

sooo, full marks to you! :drinks

Cheers,
Bel

 

Thanks Bel

Ivans little sayings have certainly stood me in good stead for the last few years!

Cornmerchant

 

Also always worth considering the more bizarrre - I had a private patient this week with basically the same symptoms. She had been given all the advice & medical help possible regarding Raynauds/chilblains. She has symptoms which are triggered by the cold but also by mild heat, which was confusing everyone. She was terrified of losing her toes or feet due to the ulceration and had finally reached the point of being fed up with the advice from Consultants (vascular, neuro, ortho, psychiatric), Gp's, Pods and every other healthcare professional she could go to and did what we all dread and 'Googled' her symptoms. As a result she requested her GP refer her to a rheumatology clinic at a London hospital which specialises in Raynauds and Scleroderma, where she was eventually diagnosed with Erythromelalgia (apparantly fairly rare). Currently taken off most of her previous meds and prescribed with Gabapentin to help with the pain - still very early days and waiting to see if any help.
Until I started talking to her about the problems and her medical history, I had looked at her feet and instantly assumed chilblains - just proves that there will always be a patient who appears to have one problem but will actually have something much more commonplace or more complicated!!!

Worth remembering that sometimes the strange looking horse is a zebra!!

(btw - she had only come in to the practice wanting a toenail cut and some advice on her dry skin!!!- shows how valuable the history taking is regardless of how simple the patients needs may initially seem).

Cheers
Rie

 

Not sure who Ivan is (Bristow or Birch perhaps?) but this saying was originally coined by Professor Ted Woodward who derived it most likely from William Ockhams principle of parsimony.

Interestingly (for geeks like me anyway) a 'zebra' is actually known medical slang for an unlikely diagnosis from ordinary symptoms.

 

Hey Rie,

Yip, they are the ones that end up in the court room.

But isn`t EM just a fancy description of symptoms, not a diagnosis? ie, Erythros (redness), melos (extremity), and algia (pain)? Much like Metatarsalgia? Tis true that many manifestations of EM are idiopathic, but presentation can often be secondary to underlying medical conditions, such as autoimmune, neurologic (hence the prescribed gabapentin or pregabalin) or blood disorders . It`s also associated with CRPS.

Now THAT is what I call music! Can`t beat a thorough med hx for clinical picture:drinks

Cheers,
Bel

 

Hi Bel,

Good points.

However, I know that as far as possible, CRPS has been ruled out, along with most other DD's (I hate myself for saying it but Wiki actually has quite a good run down of differential diagnoses!) and other linked conditions. Patient is about to undergo gentic testing along with her daughter who is beginning to suffer similar symptoms.

She is being followed up by a research group within the raynauds and scleroderma society and by the rheumatology department at a major London hospital (sorry - pt asked for discretion on detail and she is following PA now incase anyone comes up with any ideas). They certainly do not believe that EM is just a description of symptoms, once all other causes for those symptoms have been ruled out. It is classed as a condition init's pwn right.
The Gabapentin has been prescribed by the pain clinic to hopefully manage the pain symptom - she's already undergone pretty much every test available to rule out any purely neurological causes for her symptoms.

My main concern is that the patient has multiple areas of superficial ulceration, can't bear any dressings especially foam based ones, because the associated warmth triggers erythema and pain, she has skin thinner than tissue paper but thick plantar callosities in odd patches, but can't apply creams because they either cause a sensitivity reaction or the application triggers erythema and pain, and to top it all she is intolerant of many antibiotics, and it's no good mentioning insoles of any description - they trigger the same symptoms.
A basic doppler assesment found her PT and DP pulses to be tri phasic (brilliant for her age regardless of the state of her feet) and regular. Her digital pulses were found with the doppler and even her capillary refill was acceptable at 4 secs. Her feet however felt like blocks of ice. Despite digital pulses being good, her toes were mottled with erythema and cyanosis, and extremely cold but with the patient reporting them being 'boiling hot'. She has multipple digital breakdowns and ulcerations.

I am definitely not inexperienced when it comes to wound care - but how do you treat a patient who can't tolerate any dressings, insoles, antibiotics, pressure, debridement???
Any suggestions welcome...........

I've referred her from private practice into my NHS dept as I think she'll be needing treatment on a very frequent basis, and I'll then be able to get more info from the diagnosing hospital.

And yes, the history taking is totally essential, as is the writing of a thorough record of your history taking, findings and treatment (4 sides of A4 in this case).

All this for a patient who only really wanted her toenais trimmed!

I'm still however visuallising her feet and would still be willing to bet good money on visual basis that her problem was chilblains...............!!
I'll try to get some photo's.

Regards
Rie

 

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.