Erythromelalgia – Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2022 To 2032


 Erythromelalgia, a rare clinical syndrome, manifests with a triad of symptoms: redness, warmth, and burning pain, primarily affecting the extremities. Predominantly, it targets the lower extremities, notably the feet, while in some instances, it can involve the upper extremities, specifically the hands. Drenth and Michiels differentiated between erythromelalgia and erythermalgia based on their responsiveness to aspirin, resulting in three established categories:

1.       Erythromelalgia in thrombocythemia, which is platelet-mediated and responds to aspirin. This occurs in conjunction with essential thrombocytosis and polycythemia vera.

2.       Primary erythermalgia is an idiopathic or inherited disorder known as aspirin-resistant erythermalgia of unknown origin.

3.       Secondary erythermalgia, aspirin-resistant, and linked to various medical conditions.

Erythromelalgia in thrombocythemia is associated with essential thrombocytosis and polycythemia vera. Primary erythromelalgia may be either idiopathic or inherited, with the inherited form being an autosomal dominant neuropathy resulting from a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A genes. These genes encode the alpha subunits of the voltage-gated sodium channels NaV 1.7, NaV 1.8, and NaV 1.9, respectively. Secondary erythromelalgia is attributed to various underlying causes, including myeloproliferative disorders like essential thrombocytosis, polycythemia vera, and myelofibrosis, as well as infectious agents (HIV, influenza, syphilis, and poxviruses), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), and diabetes mellitus type 1 and 2. The diagnosis of erythromelalgia relies on classic signs and symptoms: redness, warmth, and burning pain, typically aggravated by heat or exercise and alleviated by cooling. Primary erythromelalgia is diagnosed based on the presence of these classic signs/symptoms and screening for mutations in the SCN9A gene. Secondary erythromelalgia is diagnosed by excluding other diseases associated with these symptoms. Pharmacological treatment and targeted neurological surgery have demonstrated effectiveness primarily in patients with secondary erythromelalgia, where treating the underlying disorder often leads to remission. Aspirin is the preferred drug for erythromelalgia associated with thrombocytopenia or myeloproliferative disorders, with other NSAIDs like anagrelide as alternatives. Managing most patients with primary and secondary erythromelalgia is challenging, and they often resist treatment. Notably, secondary disease due to polycythemia vera and essential thrombocythemia tends to respond favorably to aspirin therapy. Over time, complications such as ulceration, necrosis, and gangrene can develop due to repeated immersion in ice water or chronic cooling of the extremities. The most frequently reported complication is infection, secondary to blistering, ulceration, cyanosis, and gangrene, which can lead to amputation in chronic untreated cases. Malnutrition and hypothermia have been observed in a few instances.

·       In a population-based study in the US, the overall age- and sex-adjusted incidence rate per 100,000 people per year was 1.3.

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