From the Guidelines
Erythromelalgia should be treated with aspirin 325mg orally daily for at least 1 month as the first-line systemic treatment, given its low cost and relatively low adverse effect profile, as recommended by the most recent study 1. The treatment approach for erythromelalgia typically begins with avoiding triggers such as heat, exercise, and standing for long periods.
- First-line medications include topical agents like lidocaine 5% patches or amitriptyline-ketamine compounds.
- For systemic treatment, oral medications such as gabapentin (starting at 300mg daily, gradually increasing to 900-3600mg daily in divided doses), pregabentin (starting at 75mg twice daily, up to 300-600mg daily), or amitriptyline (10-25mg at bedtime, increasing gradually to 25-100mg) are commonly used.
- Aspirin (325mg daily) may help in cases related to platelet disorders, as it is especially effective in patients who have secondary EM due to underlying myeloproliferative disease, such as essential thrombocytosis and polycythemia vera, as reported by 1.
- For severe cases, calcium channel blockers like nifedipine (30-60mg daily) or intravenous lidocaine under medical supervision might be necessary.
- Cooling strategies such as immersing affected areas in cold water can provide immediate relief during flares, but ice and water immersion of extremities should be avoided or limited to 10 minutes at a time and 4 times daily, as suggested by 1. The condition stems from dysfunction in peripheral sensory neurons and small fiber neuropathy, causing abnormal vasodilation and pain sensation, and management is often individualized as response to treatments varies significantly between patients, and a combination approach is frequently needed for optimal symptom control, as discussed in 1.
- A step-wise approach to treatment can be followed, with aspirin as the first-line treatment, followed by gabapentin or pregabalin, and then other medications such as venlafaxine or selective serotonin reuptake inhibitors, as outlined in 1.
- Referral to a pain rehabilitation center may be necessary for patients who are recalcitrant to treatment or associated with chronic pain syndrome, as recommended by 1.
From the Research
Definition and Characteristics of Erythromelalgia
- Erythromelalgia is a rare disorder characterized by episodic burning pain with redness and warmth of the extremities 2.
- The condition is often associated with triggers such as heat, exercise, and time of day, with symptoms affecting the lower limbs in most cases 3.
- Common symptoms include burning, heat, pain, and redness, with a significant impact on quality of life 3.
Management and Treatment of Erythromelalgia
- There is no consistently effective treatment for erythromelalgia, with most recommendations based on case reports and small case series 4.
- A multidisciplinary approach to management may be needed, including patient education, avoiding episodes, relieving discomfort, and controlling secondary and underlying factors 2, 4.
- Various medications have been tried, but with limited success, including intravenous iloprost, which showed benefit in some patients 3.
- Procedural interventions, such as epidural infusion and spinal cord stimulation, have been explored, but the evidence for their use is limited 2.
Related Conditions and Treatment Options
- Erythromelalgia often co-occurs with other conditions, such as Raynaud's phenomenon and systemic sclerosis 3.
- Chronic pain management strategies, including simple analgesics, tricyclic antidepressants, and gabapentin, may be relevant to erythromelalgia treatment 5.
- Combination therapies, such as levetiracetam with gabapentin or pregabalin, have shown promise in animal models of diabetic painful neuropathy, but require further study in humans 6.