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Kalgaard OM, Clausen OP, Mellbye OJ, Hovig T, Kvernebo K. Nonspecific Capillary Proliferation and Vasculopathy Indicate Skin Hypoxia in Erythromelalgia. Arch Dermatol. 2011;147(3):309–314. doi:10.1001/archdermatol.2010.337
Erythromelalgia (EM) is a clinical syndrome that is characterized by erythema, increased skin temperature, and burning pain in the extremities. The pain is relieved by cooling and aggravated by warming.1 It is commonly divided into primary and secondary cases,2 depending on whether or not there is an underlying disease. A rare subtype, erythromelalgic syndrome (ES), which was described by Kvernebo,3 has a strong hereditary component and usually affects the skin of the feet and legs. Some authors consider EM to be a symptom complex rather than a disease entity.3,4 A clinical severity scale with 8 categories based on the need for cooling has been introduced for classification purposes.5
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