title: Clinical features and management of erythromelalgia: long term follow-up of 46 cases.
abstract: OBJECTIVE
  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.
authors: Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
authors: Louise K
authors: LK
authors: Parker
authors: Department of Rheumatology, Hospital de Santa Maria, Lisbon; and Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Lisbon Academic Medical Centre, Lisbon, Portugal.
authors: Cristina
authors: C
authors: Ponte
authors: Microvascular Diagnostics, Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK.
authors: Kevin J
authors: KJ
authors: Howell
authors: Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
authors: Voon H
authors: VH
authors: Ong
authors: Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. c.denton@ucl.ac.uk.
authors: Christopher P
authors: CP
authors: Denton
authors: Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
authors: Benjamin E
authors: BE
authors: Schreiber
mesh_terms: D000328:Adult; D004916:Erythromelalgia / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016285:Iloprost / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D011788:Quality of Life; D011928:Raynaud Disease / Q000150:complications; D012307:Risk Factors; D012907:Smoking; D063189:Symptom Assessment; D016896:Treatment Outcome
chemical_list: D010975:Platelet Aggregation Inhibitors; D016285:Iloprost