Erythromelalgia is a rare medical condition in which blood vessels,
usually in the lower extremities, are at times blocked and inflamed
Erythromelalgia disease and symptom, treatment, is it an autoimmune condition?
July 1 2015 by Ray Sahelian, M.D.
Erythromelalgia is a rare disease which involves burning pain and warmth and redness of the arms and legs. A distinction is made between primary (idiopathic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders), as well as between early- and late-onset disease.
A patient with adult
erythermalgia: evidence suggesting an autoimmune etiology.
Am J Med Sci. 2008. Jackson AL, Oates JA. Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
We report the case of a woman with a combination of erythermalgia, idiopathic thrombocytopenic purpura, and vitamin B-12 deficiency with positive parietal cell antibodies. The patient was treated with intravenous administration of immunoglobulins together with small doses of prednisone, which resulted in an improvement in her platelet counts, rise in her vitamin B12 levels, and resolution of her painful discolored digits. These findings suggest an underlying autoimmune component to the development of erythermalgia.
Late-onset erythromelalgia in a previously healthy
young woman: a case report and review of the literature.
J Med Case Reports. 2009; Gaur S, Koroscil T. Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA.
Erythromelalgia is a rare disorder characterized by episodic erythema and burning pain, which commonly involves the extremities. We present a case of late onset erythromelalgia in a previously healthy young woman and briefly review the literature. Our patient's case also has additional uncommon features not reported previously. A 33-year-old previously healthy Caucasian woman presented with complaints of episodic burning pain and flushing occurring in a central distribution involving her face, ears, upper chest and, occasionally, her upper extremities. Her symptoms were triggered by lying down or warm temperature exposure and were relieved by cooling measures. Extensive diagnostic work-up looking for secondary causes for the symptoms was negative and the diagnosis of erythromelalgia was made based on details provided in her clinical history supported by raised temperature in the affected area measured by thermography during a symptomatic episode. The patient did not respond to pharmacological therapy or surgical sympathectomy. She was advised on lifestyle modification to avoid activities which triggered her symptoms. She was hypothermic with a core temperature between 92 and 95 degrees F. She also had premature ovarian failure, which had not previously been reported. Erythromelalgia is a rare disorder of unknown cause. There is no confirmatory diagnostic test; diagnosis is based on details provided in the patient's medical history and physical examination during the episodes. For those affected, this disorder leads to significant long-term morbidity and unfortunately, to date, no definitive therapy is available except for lifestyle modification.
Most patients do not respond to medications, although occasional reports document patients as showing partial relief with lidocaine or mexiletine.
JAMA Dermatol. 2015. Topically Applied Midodrine, 0.2%, an α1-Agonist, for the Treatment of Erythromelalgia.
Curr Neurol Neurosci Rep. 2012. Pain disorders and erythromelalgia caused by voltage-gated sodium channel mutations. Voltage-gated sodium channels play a pivotal role in pain transmission. They are widely expressed in nociceptive neurons, and participate in the generation of action potentials. Alteration in ionic conduction of these channels causes abnormal electrical firing, thus renders neurons hyperexcitable. So far, mutations in the Na(v)1.7 sodium channel, which is expressed in the dorsal root ganglia cells and sympathetic neurons, have been described to cause perturbations in pain sensation. Until recently, gain-of-function Na(v)1.7 mutations were known to cause two neuropathic pain syndromes: inherited erythromelalgia and paroxysmal extreme pain syndrome. These syndromes are inherited in a dominant trait; they usually begin in childhood or infancy, and are characterized by attacks of severe neuropathic pain accompanied with autonomic symptoms. Recently, small fiber neuropathy and chronic nonparoxysmal pain have been described in patients harboring gain-of-function mutations in Na(v)1.7 channel. Loss-of-function mutations in Na(v)1.7 are extremely rare, and invariably cause congenital inability to perceive pain.
Q. I am wondering if you have any recommendations as to what natural supplements
might be helpful with erythromelalgia. As far as I know there is very little
research being done . Even though it is a very painful and often
disabling condition, it is very rare and therefore does not generate a great
interest among researchers. As far as I found out from the Erythromelalgia
Association there is some research going on in the Netherlands and also at Yale
and Mayo. I do believe China does research as well but all are short of funds.
A. I have not studied this topic to any degree but will keep my eyes open for any research regarding natural treatment with herbs, vitamins, and supplements.
Q. I have just come across your web site, trying to find medications, herbs or supplements to give me some relief with erytromelagia symptoms. At the present time I am taking Gababentin 300 mg. am. 300 mg. pm and 600 mg. with 50 mg. Amitryptoline at bedtime. I am also taking green tea extract and a multitude of vitamins. I have been to Mayo Clinic and they confirmed the erythromelalgia and basically told me to continue what I am doing. If you are familiar with erythromelalgia and have any suggestion I would greatly appreciate it.
A. We are not familiar with the nutritional treatment of this condition.
Q. I was diagnosed with erythromelalgia about 1 year
ago and I also have Raynaud's disease. Many medications have been tried without
success. I also suffer from hypertension but due to the erythromelalgia I can
not use any medications that are vasilators. I have also tried green tea extract
and propolis. I am now looking for other, natural products that might give me
some relief. I picked up a flyer at my health food store showing a product
called Nerve Shield by Redd Remedies. It contains alpha
lipoic acid, Baikal
skullcap and curcuma longa. I do not have the dosages.
I was wondering if this product could help with my erythromelalgia? I am getting desperate to find relief. The swelling, burning and turning extremely red in my feet and hand as well as ears at times seems to be getting worse, especially at night. I have to sleep in a cold room with my lower legs uncovered.
A. Sorry, but we are not familiar with the natural treatment of ereythromelalgia and don't know if the product will work.