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What Is Erythromelalgia

Erythromelalgia is a rare condition that primarily affects the feet and, less commonly, the hands (extremities). It is characterized by intense, burning pain of affected extremities, severe redness (erythema), and increased skin temperature that may be episodic or almost continuous in nature. Other names for this condition: Erythermalgia, Gerhardt disease, Mitchell disease and Weir-Mitchell disease. The diagnosis of erythromelalgia is established by a thorough evaluation of the characteristic symptoms and signs of the disease. Photographs of the affected areas during symptoms are extremely helpful (for example, photos of red feet or hands during symptoms- the redness is almost unique to erythromelalgia when associated with the history). Patient and family history can be helpful, and specialized tests may help to exclude certain disorders with similar symptoms. It is also helpful to confirm or rule out underlying diseases or conditions that may occur in association with erythromelalgia (i.e., to help differentiate primary and secondary erythromelalgia). For example, because erythromelalgia may be an early sign of certain conditions (e.g., thrombocythemia, polycythemia vera), certain laboratory tests, such as regular blood cell counts, and other specialized tests may be periodically conducted to help ensure prompt diagnosis and treatment of such underlying disorders.

Symptoms

  • Both sides of the body are usually affected (bilateral)
  • Increased skin temperature, particularly of the feet
  • Intermittent episodes of severe, burning pain associated with red hot extremities:
  • Triggering or worsening of symptoms with exposure to heat (heat intolerance) or exercise and relief with cooling.

Treatments

  • Amitriptyline
  • Avoid or reduced by elevating involved regions.
  • Calcium antagonists, magnesium selective serotonin reuptake inhibitors, tricyclic antidepressants, gabapentin or carbamazepine, antihistamines, clonazepam, misoprostol, cyproheptadine, and others
  • Exposing affected areas to cold air, such as through the use of air conditioners or fans, although again, excessive blowing air on the skin can cause its own cycle of problems (the equivalent of ‘windburn’).
  • Genetic counseling may be of benefit for people with erythromelalgia and their families
  • Ketamine
  • Lidocaine
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