Complex management of a patient with refractory primary eryt
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Erythromelalgia (EM) is a rare neurovascular disorder characterized by swelling, erythema, and burning in the extremities in a stocking-glove distribution, which is relieved by exposure to cold but worsened by heat stimuli. Based on its etiology, EM is classified into two types: primary and secondary EM. Primary EM may be either hereditary with mutations in SCN9A resulting in defective NaV1.7 sodium channels or idiopathic with spontaneous gain-of-function mutations in SCN9A or other genes. Secondary EM is associated with hematological disorders involving thrombocytosis, certain immunological disorders, and some vasoactive medications.A 41-year-old woman presented with burning and erythema in her extremities triggered by warmth and activity, which was relieved by applying ice.......

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5414616/
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