Complete Information on Erythromelalgia with Treatment and Prevention

Erythromelalgia, also known under the name of disease of the mitchell, neuralgia red, or erythermalgia, is a rare disorder in which blood vessels, usually in the lower ends, are episodically blocked and inflamed. Erythromelalgia can happen like primary or secondary disorder. Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel a-subunit gene SCN9A. Secondary erythromelalgia can result from small fiber peripheral neuropathy of any cause, hypercholesterolemia, mushroom or mercury poisoning, and some autoimmune disorders. Primary erythromelalgia may be classified as either familial or sporadic, with the familial form inherited in an autosomal dominant manner. Both of these may be further classified as either juvenile or adult onset. While the genetic cause of the juvenile and sporadic adult onset forms is often known, this is not the case for the adult onset familial form.

These symptoms are usually symmetric and affect the lower extremities more frequently than the upper extremities. Common triggers for these episodes are exertion, heating of the affected extremities, and alcohol or caffeine consumption. In some patients sugar and even melon consumption have also been known to provoke attacks. Many of those with primary erythromelalgia avoid wearing shoes or socks as the heat this generates is known to produce erythromelalgia attacks. Symptoms may remain mild for years or become severe enough to cause total disability. Generalized vasomotor dysfunction is common, and Raynaud's phenomenon may occur.

The primary method of primary management of erythromelalgia is the action to avoid releases of attack, such as heat, the with-top-effort and the consumption of alcohol. In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. Care is needed around cold water immersions, although it will provide temporary relief, it can cause many other serious problems. Frequent immersion into cold water can create a vicious cycle as the changes in temperature may cause reactive flaring. For secondary erythromelalgia, the underlying disorder is treated, aspirin may be helpful when a myeloproliferative disorder is involved. Patients respond quite variably to drug therapy and no single therapy has proved consistently effective.