Medicines for Erythromelalgia

What it is

Erythromelalgia is a rare condition that causes episodes (flare-ups) of burning pain, redness, and heat, usually affecting the feet, and sometimes the hands (or, less commonly, other areas). During a flare, the affected area becomes red, hot, and painful, with a characteristic intense burning sensation; the skin may look flushed and feel warm to the touch. The episodes are typically triggered or worsened by warmth and by things that raise the body or skin temperature — such as heat, exercise, warm rooms, wearing warm socks or shoes, or having the feet under bedclothes — and are often relieved by cooling the affected area (for example with cool water or cool air), which is a characteristic feature. The episodes can last from minutes to hours or longer, and can range from occasional and mild to frequent and severe, significantly affecting a person’s comfort, activity, sleep, and quality of life. The exact cause of erythromelalgia is not always known; it can occur on its own (primary erythromelalgia, which in some cases is inherited/genetic), or it can be associated with, or secondary to, another underlying condition — such as certain blood conditions (for example conditions affecting the blood cells or platelets), some other medical conditions, or, occasionally, medicines — so identifying any underlying cause is an important part of assessment. It is thought to involve abnormal function of the small blood vessels and nerves in the affected areas. There is no simple cure for erythromelalgia (particularly the primary form), but it can be managed: the mainstays are cooling the affected areas during flares (though care is needed, as excessive or prolonged cooling can cause skin damage), avoiding the triggers (particularly heat), treating any underlying cause, and various treatments to help control the symptoms, guided by specialists. Because it is rare and can be difficult to manage, specialist input (such as from dermatology, or other relevant specialists) is often helpful. The key messages are that erythromelalgia is a rare condition causing episodes of burning pain, redness, and heat (usually in the feet or hands), often triggered by warmth and relieved by cooling, and that cooling, avoiding triggers, and treating any underlying cause help manage it.

How it is treated

Erythromelalgia is managed by cooling the affected areas during flares (carefully), avoiding triggers, treating any underlying cause, and using treatments to help control the symptoms, guided by specialists; there is no simple cure, but it can be managed. Assessment is important, both to confirm the diagnosis and — crucially — to look for any underlying condition, as erythromelalgia can be secondary to another problem (such as certain blood conditions or others); this involves clinical assessment and tests as appropriate, and identifying and treating any underlying cause can improve or resolve the erythromelalgia, so this is a key part of care. For managing the symptoms, several approaches are used, tailored to the individual. Cooling the affected areas during a flare relieves the burning and is a mainstay — for example, cool (not ice-cold) water or cool air; however, an important caution is that excessive or prolonged cooling (such as immersing the feet in ice-cold water for long periods) can damage the skin and cause other problems, so cooling should be done carefully and not to excess, and other measures used alongside. Avoiding triggers, particularly heat, is important — such as keeping the affected areas and the body cool, avoiding warm environments, warm footwear, and heat where possible, and finding ways to keep the feet cool (for example, keeping them uncovered, or using cooling measures), while balancing this against the risk of over-cooling. Various treatments can help control the symptoms, and are guided by specialists — these can include medicines that act on the blood vessels or on nerve pain, and other approaches, chosen and adjusted for the individual, as erythromelalgia can be difficult to treat and different treatments help different people. Managing any associated conditions, and general foot and skin care (to avoid damage from the condition or from cooling), are part of care. Because erythromelalgia is rare, can be difficult to manage, and may be associated with underlying conditions, specialist input (such as from dermatology, and other relevant specialists) is often helpful, and support for the impact on quality of life (including on sleep and activity) is important. Support organisations and information can help people understand and manage this rare condition. The reassuring messages are that erythromelalgia, although a rare and sometimes difficult condition, can be managed — with careful cooling during flares, avoiding triggers (especially heat), treating any underlying cause, and treatments to help control the symptoms — and that specialist input and support help; so assessment (including for underlying causes), careful symptom management, and specialist support are the keys to managing erythromelalgia.