@article{discovery1508576,
           month = {January},
          number = {1},
            year = {2017},
           title = {Clinical features and management of erythromelalgia: long term follow-up of 46 cases},
         journal = {Clinical and Experimental Rheumatology},
          volume = {35},
            note = {{\copyright} Copyright Clinical and
Experimental Rheumatology 2017.},
           pages = {80--84},
            issn = {0392-856X},
        abstract = {OBJECTIVES: To review our clinical experience of this rare condition and describe the clinical features and response to therapy in a cohort of patients with erythromelalgia (EM), a rare condition, characterised by paroxysmal hyperthermia of the extremities with erythema, pain and intense burning. METHODS: A review was made of the electronic and paper medical records of patients with the diagnosis of EM, with a telephone interview to verify and complete clinical information relating treatment and outcome. RESULTS: 46 patients (41 females) were included in this study. Mean age was 57 years and mean duration of symptoms was 16 years. Raynaud's phenomenon was present in 36 patients (80\%) and 4 patients (9\%) had systemic sclerosis. Smoking (current or previous) was identified as a possible risk factor in 26 cases and exposure to chronic vibration in 3 cases. Overall, the effect on quality of life was mild in 15\% of cases, moderate in 30\% and severe in 48\%. The most common symptoms were burning (96\%), heat (93\%), pain (87\%), and redness (83\%). Symptoms affected the lower limbs in 98\% of cases, upper limbs in 76\%, face in 20\% and trunk in 11\%. Triggers included heat (85\%), exercise (78\%) and time of day (76\%). Various medications were tried, showing poor effect in most cases. Intravenous iloprost was given to 27 patients, with benefit in 17 patients (63\%). CONCLUSIONS: Erythromelalgia is a rare chronic debilitating condition. Exercise, heat and night time are common triggers. Current medical therapies are seldom effective and further research is sorely needed.},
          author = {Parker, LK and Ponte, C and Howell, KJ and Ong, VH and Denton, CP and Schreiber, BE},
             url = {http://www.clinexprheumatol.org/abstract.asp?a=10493}
}