Erythromelalgia: Difference between revisions

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== Background ==
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==Background==
   
* Inherited disorder that causes episodes of hot, red, painful extremities
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*Inherited disorder that causes episodes of hot, red, painful extremities
* Rare, but more common in women than men, usually starting in adulthood
+
*Rare, but more common in women than men, usually starting in adulthood
   
== Clinical Manifestations ==
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==Clinical Manifestations==
   
* Hot, red, painful extremities
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*Hot, red, painful extremities
* More commonly involves feet, but can involve hands
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*More commonly involves feet, but can involve hands
* May be associated with [[myeloproliferative disorders]] including [[essential thrombocytosis]], [[chronic myelogenous leukemia]], [[polycythemia vera]], and [[myelofibrosis]]
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*May be associated with [[myeloproliferative disorders]] including [[essential thrombocytosis]], [[chronic myelogenous leukemia]], [[polycythemia vera]], and [[myelofibrosis]]
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  +
== Management ==
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* Non-pharmacologic
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** Identify and avoid precipitants, such as heat and exercise
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** Cool with water for short periods (5 to 10 minutes every 1 to 2 hours)
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** Cool with a fan for short periods (5 to 10 minutes every 1 to 2 hours)
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** Limb elevation
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** Avoid applying ice or very cold water directly to the skin
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* Pharmacologic
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** No clear benefit but many have been tried
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** Topical treatments
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*** For pain, can try lidocaine patches, compounded amitriptyline and ketamine, capsaicin, topical diclofenac gel, gabapentin ointment
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*** For erythema, can try midodrine
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** Systemic treatments
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*** Aspirin, especially if concomitant [[Myeloproliferative disorders|myeloproliferative disorder]]
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*** For neuropathic pain, [[gabapentin]], [[pregabalin]], [[venlafaxine]], [[amitriptyline]], [[misoprostol]], [[sertraline]], [[mexiletine]], [[carbamazepine]], [[diltiazem]], [[sodium nitroprusside]], high-dose[[magnesium]]
   
 
[[Category:Dermatology]]
 
[[Category:Dermatology]]

Revision as of 14:18, 28 March 2021

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

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