Rare disease leaves skin hot, painful
DEAR DOCTOR: Will you please explain erythromelalgia? What can ease the pain other than pills?
DEAR READER: Erythromelalgia is a poorly understood and complex disease. Typical symptoms are red, hot and painful skin, typically affecting the legs or the arms. The redness and extreme burning sensation can spread up the legs to the thighs.
Erythromelalgia is quite rare, affecting only 1 or 2 people per 100,000 every year. It’s twice as common in women than men and has an average onset of occurrence at about age 55. The disease appears to have both a neurologic cause related to nerve dysfunction and a vascular cause. Even more rarely, a genetic cause can play a role. A Mayo Clinic study found that 50 percent of patients with erythromelalgia had a history of smoking, so lifestyle may be a factor as well.
Episodes can last from minutes to days — only about 3 percent of patients have continuous symptoms — and are often precipitated by an increase in temperature or by exercise. For 25 percent of patients, the symptoms are worse at night. Of note, the pain completely subsides
between episodes, so much so that in twothirds of patients, the skin is cold to the touch and takes on a purplish appearance.
Treatment often amounts to avoiding circumstances that can bring on the symptoms, such as heavy exercise and excessive heat.
Lidocaine, used as an anesthetic ointment or patch, can provide some pain relief, as can topical medications that constrict blood flow, such as brimonidine and midodrine, and topical gabapentin, capsaicin and the anti-inflammatory diclofenac.
Aspirin can reduce both pain and episode frequency when erythromelalgia is associated with bone marrow disorders, but it also has shown benefit in preventing an episode. A few reports support the use of oral steroids, such as prednisone, during an outbreak. Case reports suggest that medications used for nerve pain can decrease episodes.
Talk to your doctor about managing the condition with medication. The disease may be poorly understood, but that doesn’t mean it’s cause for hopelessness.