Erythromelalgia is a rare disorder that causes burning pain, redness, warmth, and swelling, most often in the feet or hands. Symptoms are worsened by heat and relieved by cold. The condition may be primary (often inherited) or secondary to other conditions, as part of autoimmune diseases, as an effect of certain blood disorders (such as polycythemia vera or essential thrombocythemia) or even as side effect of certain medications. The cause is believed to be a disturbance in the communication of the co-localized smallest blood vessels and sensory nerve fibers in the skin. Treatment focuses on relieving symptoms, often with cooling, pain medications, and sometimes drugs that affect blood flow or nerve signals.
In the US, between 43,000 and 70,000 individuals* are estimated to have Erythromelalgia (rare disorder (orphan) = <200,000 patients). The disease usually begins in adolescence or later in life, but may start as early as 3–4 years of age.
Erythromelalgia is a very painful condition characterized by burning pain, which occurs in sudden attacks triggered by heat, either in the environment or locally. These attacks can last for several hours and recur from a few times a week to several times a day. Patients often describe the pain as if the skin is “on fire”.
Cooling the painful area is the most effective relief, as despite the frequent trials of many drugs, there is no established effective treatment. Frequent or prolonged cooling as a treatment often leads to severe frostbite and infection, which can be life-threatening.
The disease greatly affects quality of life. Walking, standing or just being in warm environments or wearing shoes that get hot can be unbearable. Many have difficulty managing school education or their jobs, due to their frequent pain attacks, and experience sleep problems and suffer from isolation.
AlzeCure’s leading pain program, ACD440, has recently been granted Orphan Drug Designation for Erythromelalgia in the US by the FDA, as well as in Europe by the European Medicines Agency (EMA). Read more about the program here: https://www.alzecurepharma.se/en/acd440/
*References:
Sidiq SA, Asad U, Ren V. Prevalence of erythromelalgia in the United States: a cross-sectional study using the All of Us database. Arch Dermatol Res. 2024 Sep 27;316(9):646.
Reed KB, Davis MD. Incidence of erythromelalgia: a population-based study in Olmsted County, Minnesota. J Eur Acad Dermatol Venereol. 2009 Jan;23(1):13-5.