Erythromelalgia

[Guide]

image courtesy of the TEA non profit organization

Erythromelagia (EM) is one possible diagnosis to differentiate from rosacea. What is it? According to the TEA web site:

"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.

The term erythromelalgia describes the syndrome: erythros (redness), melos (extremity) and algia (pain). An alternate name is “erythermalgia” that emphasizes the thermos (heat) – an essential part of the syndrome.

While usually affecting the lower extremities (legs and feet) and upper extremities (arms and hands) other body parts like faces or just ears or the nose may be involved. It usually affects both sides of the body, but can affect just one.

The associated pain and burning sensations can be extremely severe. People with EM often make major adjustments to their lifestyles to avoid flare-ups. Even in mild-to-moderate cases, normal functioning such as walking, standing, working, socializing, exercising, and sleeping may be impaired." What is EM?

"In 2004 erythromelalgia became the first human disorder in which it has been possible to associate an ion channel mutation with chronic neuropathic pain, when its link to the SCN9A gene was initially published in the Journal of Medical Genetics." Wikipedia

EM can involve the face.[5]

Diagnosis
"Experts indicate that the intermittent nature of erythromelalgia in some cases may potentially lead to difficulties or delays in its diagnosis. Therefore, because symptoms may be reduced or absent until late in the day, for example, physicians may recommend that affected individuals take pictures of the involved regions during flaring and/or schedule clinical examinations late in the day if possible. In addition, during diagnostic assessment, physicians may possibly recommend exercise or immersion of an affected region in hot water for a certain period (e.g., approximately 10 to 30 minutes), to provoke a flare so a diagnosis may be made." [7]

Treatmentt
"In this informal survey, the use of high oral doses of magnesium produced good and sometimes dramatic results in 8 of 13 patients who had been unresponsive to many other treatments. These results suggest a possible role for high-dose oral magnesium in the treatment of EM and, perhaps, other vascular disorders." [1]

"We tried oral mexiletine, a class Ib antiarrythmic agent, in view of its reported role in various chronic painful conditions. Dramatic improvement was observed with its use. Both patients improved after several weeks of use, and there were fewer soaking episodes. We observed no adverse effects with mexilitine therapy." [2]

"Lumbar sympathetic block and TSB are useful methods for treatment of primary erythromelalgia." [3]

"We conclude that thalamic stimulation was successful in this case of primary erythromelalgia." [4]

Ketamine 0.5% and Amitriptyline 1% has been used as a treatment not only for EM but also for rosacea. 

"Even when the correct diagnosis of erythromelalgiais made, treatment is difficult, with no single therapy consistently effective." [5]

"One clinical study has demonstrated the efficacy of IV lidocaine or oral mexilitine, though differences between the primary and secondary forms were not studied. Another trial has shown promise for misoprostol, while other have shown that gabapentin, venlafaxine and oral magnesium may also be effective, but no further testing was carried out as newer research superseded this combination. Strong anecdotal evidence from EM patients shows that a combination of drugs such as duloxetine and pregabalin is an effective way of reducing the stabbing pains and burning sensation symptoms of erythromelalgia in conjunction with the appropriate analgesia. In some cases, antihistamines may give some relief. Most people with erythromelalgia never go into remission and the symptoms are ever present at some level, whilst others get worse, or the EM is eventually a symptom of another disease such as systemic scleroderma." [6]

Support

EM Thread at RF

EM Yahoo Group

TEA

We have a post on The Erythromelalgia Association [TEA] and give high marks to this non profit on how it spends its donations. If only the members of the RRDi would follow the example of TEA. We can only hope our members see the need to volunteer and make donations for rosacea as TEA members have done. If not, you can always have the other non profit rosacea organizations who are run by non rosaceans and follow how these other non profit organizations spend their donations. 

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End Notes

[1] Ann Pharmacother. 2002 Feb;36(2):255-60.
High-dose oral magnesium treatment of chronic, intractable erythromelalgia.
Cohen JS.

[2] Ann Saudi Med. 2009 Jul-Aug;29(4):316-8.
Experience with oral mexiletine in primary erythromelalgia in children.
Iqbal J, Bhat MI, Charoo BA, Syed WA, Sheikh MA, Bhat IN.

[3] Masui. 1989 Mar;38(3):388-93.
[A case of primary erythromelalgia (erythermalgia) treated with neural blockade].
Takeda S, Tomaru T, Higuchi M.

[4] Neurosurgery. 2005 Oct;57(4 Suppl):E404; discussion E404.
Thalamic stimulation as a treatment for primary erythromelalgia: technical case report.
Delye H, Lagae L, Vermylen J, Nuttin B.

[5] Dermatol Online J. 2017 Apr 15;23(4):
Erythromelalgia involving the face.
Gilmore RR, Applebaum DS, Parsons JL, Hsu S

[6] Erythromelalgia, Wikipedia

[7] Erythromelalgia, NORD