Erythromelalgia is a rare, chronic pain condition that causes episodes of burning pain, redness, and increased warmth, typically affecting the feet and hands. These episodes are often triggered by heat or physical activity and relieved by cooling. Although uncommon, the disorder can have a profound impact on daily functioning and quality of life. 

This article offers a clear and reader-friendly overview of erythromelalgia, including its causes, symptoms, diagnosis, and treatment strategies. It also outlines key differences from other similar conditions and offers practical tips for managing flare-ups. 

Erythromelalgia is a rare disorder that causes burning pain, redness, and warmth in the limbs—usually the feet and hands. These flare-ups can range from mildly uncomfortable to severely disabling and are often brought on by exposure to heat or physical activity. 

The condition is recognized by a classic triad of symptoms: 

• Burning pain 

• Redness 

• Increased warmth in the affected area 

The condition most often affects the feet and hands, although it may occasionally involve other areas such as the ears or face. Symptoms can appear suddenly and last from minutes to hours. In some cases, they progress to continuous pain, making early recognition and appropriate care essential. People with erythromelalgia may resort to extreme cooling methods, such as ice water immersion, to relieve symptoms—sometimes leading to complications like frostbite or skin breakdown. 

Erythromelalgia is rare, with estimated prevalence ranging from 0.36 to 1.3 cases per 100,000 people. It may be underdiagnosed due to similarities with other pain disorders and lack of awareness among healthcare providers. 

There is a bimodal age distribution: 

• Primary erythromelalgia: More common in children and young adults 

• Secondary erythromelalgia: More often diagnosed in adults over 40 

Women appear to be affected more frequently than men, and familial cases involving gene mutations are less common but may have a more severe course. 

Erythromelalgia is broadly classified into two main types: primary and secondary. 

Primary erythromelalgia can be: 

• Idiopathic, with no known cause 

• Familial, often linked to a mutation in the SCN9A gene, which affects sodium channels in sensory nerves 

Secondary erythromelalgia is associated with other medical conditions, including: 

• Myeloproliferative disorders like essential thrombocythemia and polycythemia vera 

• Autoimmune diseases such as lupus 

• Diabetes 

• Peripheral neuropathies 

• Medication side effects, including from calcium channel blockers or bromocriptine 

In primary erythromelalgia, genetic changes often lead to hypersensitivity in the nerves. In secondary forms, underlying illnesses trigger abnormal blood flow or nerve responses. 

Erythromelalgia is thought to result from a combination of vascular and nerve dysfunction. Some of the key mechanisms include: 

• Microvascular abnormalities causing increased blood flow and tissue warmth 

• Nerve fiber dysfunction, particularly involving small pain-sensing fibers 

• SCN9A gene mutations, leading to overactive pain signals 

• Platelet clumping or microvascular occlusion in patients with blood disorders 

• Inflammatory responses that promote redness and pain 

These factors may work in tandem, creating a vicious cycle of heat sensitivity, vasodilation, and burning discomfort. 

The hallmark signs and symptoms of erythromelalgia revolve around the classic triad of burning pain, redness, and warmth. These symptoms typically appear in the feet and hands and may also affect the ears or face. The pain can be throbbing, stinging, or stabbing, and is often accompanied by visible swelling during an episode. 

Symptoms usually affect both sides of the body symmetrically. Attacks can last from several minutes to a few hours, with some patients experiencing episodes daily, while others may have them less frequently. After an attack, the skin may appear discolored or bluish. Some people also experience excessive sweating or even skin ulcers, especially if prolonged cooling is used. 

Common triggers for erythromelalgia include warm weather, exercise, tight shoes, and emotional stress. Relief is often found by exposing the affected area to cool air, elevating the limbs, or resting in a cooler environment. 

Several medical conditions share overlapping features with erythromelalgia. Accurate diagnosis depends on careful history and examination. Common look-alikes include: 

• Cellulitis – usually one-sided, with fever and signs of infection 

• Peripheral neuropathy – nerve pain without warmth or redness 

• Complex regional pain syndrome (CRPS) – includes color changes and skin sensitivity 

• Acrocyanosis – blue, cold extremities without pain 

• Chilblains (pernio) – cold-induced red or purple skin lesions 

• Gout – localized joint inflammation, especially in the big toe 

• Raynaud’s phenomenon – color changes triggered by cold, not heat 

Erythromelalgia is primarily diagnosed based on symptoms and physical findings. There is no single test that confirms the condition. 

Doctors rely on: 

• A history of heat-triggered, burning pain in the extremities 

• Physical exam during or after episodes (when symptoms are visible) 

• Exclusion of other conditions with similar presentations 

Supportive tests may include: 

• Blood work to screen for myeloproliferative or autoimmune disorders 

• Nerve studies to assess for small fiber neuropathy 

• Skin biopsy in selected cases 

• Genetic testing if a familial cause is suspected 

• Thermography to detect elevated skin temperatures during flares 

While there is no cure, erythromelalgia can often be managed with a combination of lifestyle adjustments and medications. Treatment is individualized, depending on symptom severity and whether the condition is primary or secondary. 

Lifestyle Strategies 

• Avoid exposure to heat and triggers 

• Use fans or cool water for gentle skin cooling 

• Choose breathable footwear and socks 

• Practice safe cooling (avoid ice baths) 

• Keep a pain diary to identify patterns 

Medications 

For blood-related cases: 

• Aspirin (especially if linked to high platelet counts) 

For nerve-related pain: 

• Gabapentin or pregabalin 

• Duloxetine or venlafaxine 

• Carbamazepine or oxcarbazepine 

To reduce nerve excitability: 

• Mexiletine (oral) 

• Lidocaine (topical or IV infusions in hospitals) 

Topical therapies: 

• Lidocaine patches 

• Capsaicin cream 

• Amitriptyline cream 

Interventional Approaches 

In rare, severe cases that don’t respond to medications: 

• Sympathetic nerve blocks 

• Spinal cord stimulation 

Addressing Underlying Causes 

In secondary erythromelalgia, treating the underlying condition often leads to symptom improvement. This may involve blood pressure medications, immune therapy, or blood cell count management, depending on the root cause. 

When not properly managed, erythromelalgia can lead to: 

• Skin ulcers or breakdown from excessive cooling 

• Infections due to impaired skin integrity 

• Mobility issues from pain and swelling 

• Sleep problems from nighttime flare-ups 

• Mental health effects, including anxiety and depression 

While erythromelalgia can’t always be prevented, patients can take proactive steps to reduce flare-ups: 

• Learn personal triggers and avoid them when possible 

• Use safe cooling strategies 

• Stay hydrated and maintain good skin hygiene 

• Treat any underlying health conditions 

• Work with a multidisciplinary team for support 

The outlook for erythromelalgia varies widely: 

• Some individuals experience episodic symptoms that improve over time 

• Others may have persistent discomfort despite treatment 

• Secondary forms often improve when the root condition is treated 

Although complete remission is rare, many people can achieve meaningful symptom relief with proper management and lifestyle adaptation. 

Erythromelalgia is a complex, painful disorder marked by episodes of burning, red, and hot extremities. Though rare, it can significantly disrupt daily life and requires a thoughtful, personalized approach to diagnosis and care. 

Managing erythromelalgia involves identifying triggers, using appropriate medications, and treating any underlying conditions. With education, support, and a tailored treatment plan, many individuals can lead active, fulfilling lives despite the challenges posed by this condition. 

1. Davis MD, O’Fallon WM, Rogers RS, Rooke TW. Natural history of erythromelalgia: Presentation and outcome in 168 patients. Arch Dermatol. 2000;136(3):330–336. 

2. Thompson GH, Hahn G, Rang M. Erythromelalgia. Clin Orthop Relat Res. 1979;(144):249–254. 

3. Klein CJ, et al. Inherited erythromelalgia: novel SCN9A mutations and variable sodium channel dysfunction. Ann Neurol. 2013;74(6):885–894. 

4. Cohen JS. Erythromelalgia: New theories and new therapies. J Am Acad Dermatol. 2000;43(5 Pt 1):841–847. 

5. Mork C, Kvernebo K. Erythromelalgia: A mysterious condition? Arch Dermatol. 2000;136(3):406–409. 

6. Mann N, King T. Erythromelalgia: A review of current knowledge. J Vasc Surg. 2019;69(6):2152–2159. 

7. The Erythromelalgia Association (TEA). Available at: www.erythromelalgia.org