Erythromelalgia

Background

• Inherited disorder that causes episodes of hot, red, painful extremities
• Rare, but more common in women than men, usually starting in adulthood

Clinical Manifestations

• Hot, red, painful extremities
• More commonly involves feet, but can involve hands
• May be associated with myeloproliferative disorders including essential thrombocytosis, chronic myelogenous leukemia, polycythemia vera, and myelofibrosis

Management

• Non-pharmacologic
  • Identify and avoid precipitants, such as heat and exercise
  • Cool with water for short periods (5 to 10 minutes every 1 to 2 hours)
  • Cool with a fan for short periods (5 to 10 minutes every 1 to 2 hours)
  • Limb elevation
  • Avoid applying ice or very cold water directly to the skin
• Pharmacologic
  • No clear benefit but many have been tried
  • Topical treatments
    • For pain, can try lidocaine patches, compounded amitriptyline and ketamine, capsaicin, topical diclofenac gel, gabapentin ointment
    • For erythema, can try midodrine
  • Systemic treatments
    • Aspirin, especially if concomitant myeloproliferative disorder
    • For neuropathic pain, gabapentin, pregabalin, venlafaxine, amitriptyline, misoprostol, sertraline, mexiletine, carbamazepine, diltiazem, sodium nitroprusside, high-dosemagnesium