Disorder characterized by vasodilatation associated with paroxysmal, intense burning pain and episodic reddening of the extremities (mainly feet). The clinical symptoms include redness, heat, pain, and swelling. Typically, it affects both sides of the body, it may sometimes be unilateral. The primary form is present when the manifestations are not associated with an organic disease and it is usually called “erythermalgia” because of the significance of the heat felt in the extremities involved. The secondary or acquired form is related to underlying medical conditions. The clinical hallmarks include triggering or worsening of symptoms with exposure to heat or exercise and relief with cooling. The semantic of the word refers to the prefix “erythro-” that means redness, “mel-” indicates the limbs, and the suffix “-algia” refers to the associated pain.
Acromelalgia; Erythermalgia; Gerhardt Disease; Weir-Mitchell Disease; Mitchell disease.
There are two types of erythromelalgia described:
Primary Familial Erythromelalgia: Classified either familial or sporadic, inherited as an autosomal dominant trait. A juvenile or adult onset is suggested as subclassification. The juvenile onset occurs before the age of 20 and frequently prior to age 10. While the genetic cause for the juvenile and sporadic adult onset forms is established, it is not the case for the adult onset familial form.
Secondary Familial Erythromelalgia: Characterized by the presence of small fiber peripheral neuropathy that can be associated with polycythemia vera, essential thrombocytosis, hypercholesterolemia, mercury or fungi (Clitocybe Acromelalgia in Japan; and Clitocybe Amoenolens in France) poisoning and autoimmune disorders. The mushroom-induced erythromelalgia has been reported to last between 8 days and 5 months.
The first case was reported in 1834 by Richard James Graves (1796-1853), the eminent Irish surgeon. Dr Graves was President of the Royal College of Physicians of Ireland, Fellow of the Royal Society of London and the co-founder of the Dublin Journal of Medical Science. The American physician Silas Weir Mitchell (1829-1914) suggested the term “Erythromelalgia” in 1878, and the German physician Carl Jakob Gerhardt (1833-1902) provided further insights into erythromelalgia in 1892.
The incidence of EM in the world has been estimated (based on only few studies) at 4.7:100,000 people with a mean of 1:3.7 male to female ratio. It was determined in a 1997 report from Norway that the estimated annual incidence was 2:100,000, with a 1:2.4 male to female ratio, respectively. In the United States, a population-based study in 2009 reported that the incidence of the disease in the Olmsted County, Minnesota, was 1.3:100,000, with a 1:5.6 male to female ratio, respectively. In this report, the incidence between primary and secondary EM was 1.1 vs 0.2 per 100,000 people per year, respectively. In the south of Sweden, the most recent reported overall annual population-based incidence was estimated at ...