J Mal Vascul
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Several classifications have been proposed for erythermalgia according to causality (primary or secondary) or age of onset (presuming that all early-onset are primary). Recently a classification in three types of erythromelalgia have been proposed. Erythromelalgia and erythermalgia are defined as two independent and completely different disease entities. ⋯ We recently observed 19 cases of erythermalgia; nine had primary erythermalgia; in 6 of 9, symptoms were relieved with aspirin. Ten of them had a secondary erythermalgia: 5 due to myeloproliferative disorders (erythromelalgia), 2 systemic lupus erythematosus and 3 to drugs. We used a two-level classification with a first level of primary or secondary erythermalgia, and a second level for primary erythermalgia, of familial or nonfamilial primary and for secondary erythermalgia, of thrombocythaemia disorders or other.
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We report three cases of erythermalgia associated with systemic lupus erythematosus corresponding to different clinical situations in such an association. The first patient developed erythermalgia during the course of systemic lupus erythematosus. In the second, erythermalgia preceded other symptoms of systemic lupus erythematosus by four years. ⋯ These cases permit a discussion on terminology and classification of erythromelalgia and erythermalgia. However, more than terminology or classification into three types or into adult-onset and early-onset (childhood) erythromelalgia, the important is to consider primary and secondary forms. We used a classification into two types: primary (or erythermalgia) with subdivision into sporadic and familial subtypes, and secondary with subdivision into erythermalgia related to myeloproliferative disorders and erythermalgia related to other diseases, such as systemic lupus erythematosus, or to drugs (erythermalgia-like syndrome).