Who typically treats erythromelalgia?

Management of Erythromelalgia Typically Requires a Multidisciplinary Team Approach

Erythromelalgia patients typically benefit most from a multidisciplinary team that includes physicians in dermatology, neurology, vascular medicine, and pain medicine, with additional specialists based on underlying causes. 1

Core Multidisciplinary Team Members

• Dermatology: Often the first point of contact due to skin manifestations (redness, warmth)
• Neurology: Essential for patients with small or large fiber neuropathy and for management of neuropathic pain components 1
• Vascular Medicine: Addresses the vascular dysregulation aspects of the condition
• Pain Medicine: Critical for pain management strategies and rehabilitation approaches 1

Specialist Additions Based on Underlying Causes

• Hematology: Essential for patients with underlying myeloproliferative disease 1, 2
• Oncology: Valuable when managing underlying myeloproliferative disorders 1
• Comprehensive Pain Rehabilitation: For severe, refractory cases with chronic pain syndrome 1

Treatment Approach Algorithm

1. Initial Assessment and Diagnosis:

   • Determine if primary (idiopathic/inherited) or secondary (with identifiable cause)
   • Screen for myeloproliferative disorders with blood counts (particularly important as erythromelalgia can precede diagnosis by median of 2.5 years) 2

2. First-Line Management:

   • Lifestyle modifications to avoid triggers
   • Topical treatments for pain (lidocaine patches/cream) and redness (compounded midodrine 0.2%) 1, 3
   • Aspirin 325mg daily (particularly effective for secondary erythromelalgia due to myeloproliferative disorders) 3, 2

3. Second-Line Management:

   • Systemic medications based on pain characteristics
   • Sodium channel blockers for primary/inherited erythromelalgia
   • Treatment of underlying cause for secondary erythromelalgia

4. Refractory Cases:

   • Comprehensive pain rehabilitation program 1
   • Consider procedural interventions (though evidence is limited) 4

Treatment Effectiveness Considerations

• Secondary erythromelalgia due to myeloproliferative disorders: Responds dramatically to aspirin therapy 5, 2
• Primary/inherited erythromelalgia: Often more resistant to treatment, especially childhood-onset forms 5
• Chronic pain syndrome: May require comprehensive pain rehabilitation programs 1

Important Cautions

• Avoid excessive cooling measures: Limit ice/water immersion to prevent tissue damage 1, 3
• Avoid opioids: Not considered appropriate for long-term management due to risks and addictive potential 1, 3
• Monitor for complications: Patients may develop severe disability without proper management 1, 6

The Mayo Clinic experience demonstrates that a multidisciplinary approach with comprehensive pain rehabilitation can dramatically improve outcomes, with documented cases of patients progressing from wheelchair dependence to resuming normal activities including sports 1.