Erythromelalgia
What is the cause?
For most people witherythromelalgia,the cause is unknown.
However, in some cases, an underlying cause may be found usually a medical condition or a faulty gene (see below).
Underlying disease
Erythromelalgia sometimes results froman underlying disease, such as:
- havingabnormally high levels ofblood cells either havingtoo manyplatelets in the blood (thrombocythemia) or too manyred blood cells ( Polycythaemia )
- damage to the peripheral nervous system thenetwork of nerves outside the brain and spinal cord (this damage is known as peripheral neuropathy )
- multiple sclerosis (MS) a disease of the nerves in the brain and spinal cord
- anautoimmune problem such as lupus or rheumatoid arthritis , where the immune system attacks the body's own tissues
It may also result from certain medications, such as verapamil or nifedipine.
Genetic cause
In somepeople with erythromelalgia, the disease is caused by a change (mutation) in a gene. This may have occurred spontaneously or been inheritedfrom a parent, meaning that erythromelalgia can run in the family.
Genes contain the genetic information, or the "instructions", to make a particular protein in the body. Genetic mutations occur when DNA changes, altering the genetic instructions.
Erythromelalgia can be caused by changes in the SCN9A gene thatprovides the instructions for a sodium channel in pain nerves.
Changes in sodium channels can lead to them opening more easily and staying open longer than usual. This increases or amplifies pain signals.
It is not currently understood why the hands and feet are affected the most.
Medically Reviewed by a doctor on 21 Dec 2018
Introduction
Erythromelalgia is a rare disorder that causes episodes of burning pain and redness in the hands and feet, and sometimes the arms, legs, ears and face.
How severe can it be?
People with erythromelalgia typically suffer episodes or flare-ups of severe pain lasting from a few minutes to days. Flare-ups usually start as an itching sensation, which worsens to a severe burnin
What are the triggers?
Symptoms are usually triggered by an increase in body temperature. They can start after exercising,wearing warm socks, gloves or tight shoes,entering a warm room, being dehydrated, or evenconsuming a
What is the cause?
For most people witherythromelalgia,the cause is unknown. However, in some cases, an underlying cause may be found usually a medical condition or a faulty gene (see below). Underlying disease Eryt
Can it be treated?
Cooling the skin and elevation Cooling the skin with a fan or cold water, or raising the affected hands or feetmay relieve the pain. However, using ice or soaking the hands or feet for long amounts
Information about you
If you or your child haserythromelalgia, your clinical team will pass information about you/your childon to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS). This help
More information and support
Specialist centres in the UK Paediatric cases: Great Ormond Street Hospitalfor Children Pain Control Service Clinical Lead for Pain Management Department of Anaesthesia and Pain Medicine Level 4, P
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