EM can be subdivided into four distinct entities?

Hi Guys,

Came across this in one of my research journals.

" It appears that EM can be subdivided into four distinct entities: two predominantly affecting adults and two usually found in children".

Have attached the paper for you to read ( see discussion ).

Any thoughts on this ?

God bless


316-Erythromelalgiaanendothelialdisorderresponsivetosodnitroprusside.pdf (110 KB)

Hi mads I can’t seem to get the paper. Can you send the link?Thank you!

I looked for the paper and couldn’t find it?

Hi Guys,

Paper is attached . Its in the discussion on sod nitroprusside "It appears that EM can be subdivided into four distinct
entities: two predominantly affecting adults and two usually
found in children"

However- others say 3 types, and 2 types.

No consensus , no wonder they have problems diagnosing us ;)


317-3typesofEM.pdf (501 KB) 318-HereditaryandacquiredarticleEM2types.pdf (116 KB) 319-Erythromelalgia.DermNetNZ.htm (17.8 KB)

WOW this is so great to put this available to us! Thanks Mad!

"Erythromelalgia may be an early sign of polycythaemia or thrombocythaemia, where symptoms may precede diagnosis of the myeloproliferative disorder by 2.5 years. Dramatic relief with aspirin is typical of this type and can be used as an aid to diagnosis."

This is the interesting part for me. Eg: Today I took a baby aspirin. Few hours later my EM is not bad at all. I can stand on carpet, etc without flaring...

Does anyone else have positive effects with aspirin? How long have they had EM? Do they have a MPN?

(Thanks in advance)

Yes- I have positive effects with aspirin Ocker. I started taking aspirin from the start . Ill send you some stuff on aspirin - theoretically makes sense and OTC. Dosage is up to 350mg day - thats what most research tends to recommend.

What type of myeloproliferative neoplasms (MPN) do you have? Have you been diagnosed?

Have you tried out antihistamines - another good rationale. EM - biopsy- heat intolerance- chronic urticaria .

God bless


Thank you for the research Mads.i really appreciate all of the work you do finding the research to help us all.

Hi Mads

I don’t have an MPN as yet - diagnosed about Aug last year. Last blood tests were within normal ranges.

I’m concerned about my condition moving to a MPN and thus the curiosity as to others here that respond to Aspirin and if it developed to a diagnosed MPN… Doc says that if you respond to aspirin that it does point to an MPN but could take some time to develop…

Thanks too by the way for any info… Much appreciated

The paper was interesting and made me grateful I fall into the secondary and delayed on set Em grouping. My heart just goes out to children in pain. Thanks for sharing!

Does anyone have more information on the triggers?