Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness, predominantly involving the extremities. The feet are more frequently involved than the hands. Rarely, non-extremity involvement is seen in areas such as the face and genitals.
A distinction is generally made between primary (idiopathic or genetic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders), as well as between early- and late-onset disease.
There are a few types and subtypes of erythromelalgia, each with its own causes. These types include:
Primary erythromelalgia occurs in isolation without any underlying diseases. There are two primary types:
Idiopathic: The most common form of erythromelalgia. It occurs with no known cause.
Inherited: A defective gene is passed down from parent to child.
Secondary erythromelalgia results from an underlying condition relating to neurological, blood, or immunological disorders. These conditions include:
The pain from erythromelalgia can come and go. These are known as flares or episodes. Flares are more common in the evening and night.
Flares often involve painful, burning areas of skin that are also:
Flares can be made worse in warm conditions, caused by:
There’s no specific test for diagnosing erythromelalgia. Your healthcare provider reviews your symptoms and examines any active flare-ups or photos of flare-ups. Multiple tests may be necessary to confirm your symptoms and rule out other medical issues. These include:
Note: For purposes of this section, a characteristic attack of erythromelalgia consists of burning pain in the hands, feet, or both, usually bilateral and symmetrical, with increased skin temperature and redness, occurring at warm ambient temperatures. These evaluations are for the disease as a whole, regardless of the number of extremities involved.
Characteristic attacks that occur more than once a day, last an average of more than two hours each, respond poorly to treatment, and that restrict most routine daily activities
Characteristic attacks that occur more than once a day, last an average of more than two hours each, and respond poorly to treatment, but that do not restrict most routine daily activities
Characteristic attacks that occur daily or more often but that respond to treatment
Characteristic attacks that occur less than daily but at least three times a week and that respond to treatment
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