From the Guidelines
Aspirin is the recommended first-line treatment for erythromelalgia, with a daily dose of 325mg for at least 1 month, due to its low cost and relatively low adverse effect profile, as well as its effectiveness in patients with secondary erythromelalgia due to underlying myeloproliferative disease 1.
Treatment Approach
The treatment of erythromelalgia typically involves a combination of lifestyle modifications and medications.
- Avoiding triggers such as heat, exercise, and alcohol is essential, while using cooling methods like fans or cold water immersion for symptom relief.
- First-line medications include topical treatments, but aspirin 325mg orally daily for a trial of 1 month is recommended as the first-line systemic treatment 1.
- For neuropathic pain, consider gabapentin or pregabalin, but these should be considered as second-line therapy 1.
Lifestyle Modifications
- Maintain a lifestyle that is as normal as possible
- Cool affected areas safely, avoiding ice and water immersion of extremities for more than 10 minutes at a time and 4 times daily 1
Medications
- Aspirin 325mg orally daily for at least 1 month as the first-line systemic treatment 1
- Gabapentin or pregabalin as second-line therapy for neuropathic pain 1
- Topical treatments such as lidocaine 5% patches or amitriptyline-ketamine compounds applied to affected areas 2-3 times daily 1
Monitoring and Referral
- Regular monitoring for medication side effects is essential, particularly with anticonvulsants and antidepressants.
- Referral to a pain rehabilitation center may be considered for severe cases unresponsive to other treatments 1.
From the Research
Treatment Options for Erythromelalgia
- Aspirin is a useful treatment for erythromelalgia associated with myeloproliferative disorders 2
- Sodium channel blockers such as lidocaine, carbamazepine, and mexiletine are used to treat primary erythromelalgia 2
- Topical therapies are preferred for their limited side effects and are used for those with contraindications to systemic therapies 3
- Gabapentin has been reported to be effective in treating erythromelalgia in some cases 4
- Interosseous membrane stimulation appears to be a safe, simple, and effective treatment for erythromelalgia 5
- Procedural interventions such as epidural infusion, sympathetic ganglion block, sympathectomy, pulsed radiofrequency, spinal cord stimulation, dorsal root ganglion stimulation, brain stimulation, transcranial magnetic stimulation, and botulinum toxin injections have been used to manage erythromelalgia, but the evidence for their use is limited 6
Medications Used to Treat Erythromelalgia
- Aspirin 2, 4
- Indomethacine 4
- Beta-blockers 4
- Calcium channel antagonists 4
- Misoprostol 4
- Diltiazem 4
- Tricyclic antidepressants 4
- Serotonin reuptake inhibitors 4
- Gabapentin 4
- Lidocaine 2, 4
- Carbamazepine 2
- Mexiletine 2