A 14-year-old girl presented for evaluation of 3 years of intermittent episodes of acute pain in the hands and feet. She had no other medical history. These episodes of pain were frequently triggered by physical exercise and bathing.
During physical examination, movement of the patient's right hand triggered burning pain, and the hand became acutely erythematous. Immersion of the right hand in warm water triggered the same kind of pain and erythema. The patient's presentation was typical of erythromelalgia, in which an increase in temperature triggers attacks of acute pain and red, swollen hands and feet. In some patients, pain and redness can extend beyond the distal extremities. Symptoms are relieved by exposure to cold.
Erythromelalgia can be seen in association with myeloproliferative disorders, connective-tissue disorders, vasculitis, diabetes, gout, multiple sclerosis, thrombotic thrombocytopenic purpura, and the use of certain drugs. Cases of erythromelalgia that are associated with myeloproliferative disorders usually respond to aspirin. Otherwise, treatment is difficult, although attendance at a pain rehabilitation center can be beneficial. The patient had no underlying disease, and no medical treatment was effective. She manages the erythromelalgia by avoiding triggering factors.
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