ERYTHERMALGIA AND SYSTEMIC LUPUS-ERYTHEMA TOSUS

We report three cases of erythermalgia associated with systemic lupus erythematosus corresponding to different clinical situations in such a n association. The first patient developed erythermalgia during the co urse of systemic lupus erythematosus. In the second, erythermalgia pre ceeded other symptoms of systemic lupus erythematosus by four years. I n the third, erythromelalgia was not related to a flare-up of systemic lupus erythematosus, but to thrombocythemia, a complication of immuno suppressive therapy. These cases permit a discussion on terminology an d classification of erythromelalgia and erythermalgia. However, more t han terminology or classification into three types or into adult-onset and early-onset (childhood) erythromelalgia, the important is to cons ider primary and secondary forms. We used a classification into two ty pes: primary (or erythermalgia) with subdivision into sporadic and fam ilial subtypes, and secondary with subdivision into erythromelalgia re lated to myeloproliferative disorders and erythermalgia related to oth er diseases, such as systemic lupus erythematosus, or to drugs (erythe rmalgia-like syndrome) (J Mal Vasc 1996; 21: pages 88-91).