eprintid: 1508576 rev_number: 32 eprint_status: archive userid: 608 dir: disk0/01/50/85/76 datestamp: 2017-02-03 15:24:43 lastmod: 2021-11-29 00:12:35 status_changed: 2017-02-03 15:24:43 type: article metadata_visibility: show creators_name: Parker, LK creators_name: Ponte, C creators_name: Howell, KJ creators_name: Ong, VH creators_name: Denton, CP creators_name: Schreiber, BE title: Clinical features and management of erythromelalgia: long term follow-up of 46 cases ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: G90 note: © Copyright Clinical and Experimental Rheumatology 2017. 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. date: 2017-01-26 date_type: published official_url: http://www.clinexprheumatol.org/abstract.asp?a=10493 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green article_type_text: Journal Article verified: verified_manual elements_id: 1148900 pii: 10493 lyricists_name: Denton, Christopher lyricists_name: Howell, Kevin lyricists_name: Ong, Voon lyricists_name: Schreiber, Benjamin lyricists_id: CPDEN87 lyricists_id: KHOWE18 lyricists_id: VONGX45 lyricists_id: BESCH99 actors_name: Denton, Christopher actors_id: CPDEN87 actors_role: owner full_text_status: public publication: Clinical and Experimental Rheumatology volume: 35 number: 1 pagerange: 80-84 event_location: Italy issn: 0392-856X citation: Parker, LK; Ponte, C; Howell, KJ; Ong, VH; Denton, CP; Schreiber, BE; (2017) Clinical features and management of erythromelalgia: long term follow-up of 46 cases. Clinical and Experimental Rheumatology , 35 (1) pp. 80-84. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/1508576/1/CER9395%20Parker%20et%20al%20%E2%80%93%20Clinical%20features%20and%20management%20of%20erythromelalgia.pdf