E is for Erythromelalgia

December 10, 2021

Erythromelalgia is a distressing and difficult to manage condition that affects the hands and feet and is characterized by intermittent attacks with the triad of symptoms of redness, warmth and burning pain that can last from minutes to days. It needs to be differentiated from chilblains and burning feet syndrome

Erythromelalgia is a rare condition characterized by episodes of intense burning pain, redness, and increased temperature in the extremities, particularly the hands and feet. The condition is believed to be caused by dysfunction in the peripheral nervous system, leading to abnormalities in the blood vessels and increased blood flow to the affected areas.

Symptoms: The main symptom of erythromelalgia is a sensation of intense burning pain, often accompanied by redness, warmth, and swelling in the affected areas. The pain is typically triggered or worsened by factors that increase blood flow, such as exposure to heat, warm temperatures, exercise, or elevation of the affected limbs. Symptoms may be episodic and can last for varying durations, from minutes to hours.

Pathophysiology: Erythromelalgia is thought to involve dysfunction of the small nerve fibers and blood vessels in the affected areas. This dysfunction leads to an abnormal opening of the blood vessels, resulting in increased blood flow and the characteristic symptoms. The exact cause of erythromelalgia is not fully understood, but in some cases, it can be associated with underlying conditions such as primary erythromelalgia (a genetic form) or secondary erythromelalgia, which can be caused by nerve damage, autoimmune disorders, blood disorders, or medication side effects.

Diagnosis: Erythromelalgia is diagnosed based on the characteristic symptoms and a thorough clinical evaluation. There is no specific diagnostic test for the condition, so other potential causes of similar symptoms, such as peripheral neuropathy or vascular disorders, must be ruled out.

Treatment: The management of erythromelalgia focuses on relieving symptoms and minimizing triggers. This may involve lifestyle modifications, such as avoiding triggers like heat or warm temperatures and elevating the affected limbs. Medications can also be prescribed to help control symptoms, including medications that reduce nerve activity (such as certain anticonvulsants), improve blood flow, or modulate pain signals.

Prognosis: Erythromelalgia is a chronic condition that can have a significant impact on quality of life. Symptoms can vary in severity and frequency, and there is no known cure. However, with proper management and identification of triggers, many individuals with erythromelalgia can find relief from their symptoms and experience improved quality of life.


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