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Clinical features and management of erythromelalgia: long term follow-up of 46 cases
L.K. Parker1, C. Ponte2, K.J. Howell3, V.H. Ong4, C.P. Denton5, B.E. Schreiber6
- Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
- 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.
- Microvascular Diagnostics, Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK.
- Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
- Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK. c.denton@ucl.ac.uk
- Centre for Rheumatology, Royal Free London NHS Foundation Trust, London, UK.
CER9395
2017 Vol.35, N°1
PI 0080, PF 0084
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PMID: 27494156 [PubMed]
Received: 03/03/2016
Accepted : 04/07/2016
In Press: 02/08/2016
Published: 26/01/2017
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.