What are the treatment options for erythromelalgia?

Treatment Options for Erythromelalgia

Erythromelalgia treatment should follow a stepwise approach starting with topical therapies as first-line treatment, followed by aspirin (especially for secondary erythromelalgia), and then other systemic medications if needed. 1

Initial Management

• Evaluate for underlying causes or associations (especially myeloproliferative disorders which can precede diagnosis by 2.5 years) 2
• Implement lifestyle modifications to minimize flares while maintaining normal activities 1:
  • Avoid triggers such as excessive heat and strenuous exercise
  • Limit cooling measures (ice/water immersion should be restricted to 10 minutes, maximum 4 times daily) to prevent tissue damage 1
  • Consider alternative exercise options (e.g., swimming instead of running) 1

First-Line Treatments

Topical Therapies (4-week trial for each)

For pain management:

• Lidocaine patches (4% OTC or 5% prescription) applied to affected areas for 12-24 hours 1
• Compounded amitriptyline-ketamine mixture applied to affected areas up to 3 times daily 1
• Capsaicin cream (0.025-0.075%) applied 3-4 times daily for 6 weeks (warn patients about initial burning sensation) 1

For erythema management:

• Compounded midodrine 0.2% applied 3 times daily (evidence-based preferred treatment) 1
• Alternative options include oxymetazoline 0.05%, timolol maleate 0.5%, or brimonidine tartrate 0.33% (caution about rebound erythema) 1

Second-Line Treatments

Systemic Medications (3-4 month trial for each)

• Aspirin 325mg daily for at least 1 month - particularly effective for secondary erythromelalgia associated with myeloproliferative disorders 1, 2
• Other NSAIDs (for aspirin-allergic patients): ibuprofen, indomethacin, naproxen, piroxicam 1
• Sodium channel blockers:
  • Mexiletine - particularly useful for primary erythromelalgia 3, 4
  • Carbamazepine 3
• Corticosteroids - consider early in disease course before irreversible nociceptive remodeling 1

Third-Line Treatments

• Referral to a comprehensive pain rehabilitation center for multidisciplinary management 1
• Consider specialized infusion therapies in refractory cases:
  • Intravenous lidocaine (note: safety concerns exist including neurologic effects) 1
  • Ketamine infusion (limited evidence, controversial due to adverse effects) 1

Special Considerations

• For secondary erythromelalgia due to myeloproliferative disorders, treatment should focus on addressing the underlying condition 1
• Primary familial erythromelalgia (associated with SCN9A gene mutations) may respond better to sodium channel blockers 3
• Monitor all adult patients with erythromelalgia with periodic blood counts, as symptoms often precede myeloproliferative disorders by years 5
• Topical therapies are generally preferred due to limited side effects compared to systemic options 6

Common Pitfalls to Avoid

• Excessive cooling with ice water or fans can lead to tissue damage, ulcerations, and worsening symptoms when discontinued 1
• Delaying treatment of underlying myeloproliferative disorders in secondary erythromelalgia 2
• Failing to try aspirin in adult patients, which can provide dramatic relief in many cases 5
• Discontinuing treatments too quickly before adequate trial periods (4 weeks for topicals, 3-4 months for systemic medications) 1