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Neurophysiologic and vascular studies in erythromelalgia: a retrospective analysis
P Sandroni1, M D Davis, C M Harper
1From the *Department of Neurology, daggerDepartment of Dermarology, the double dagger Section of Biostatistics, and the Gonada Vascular Center, Mayo Clinic and Mayo foundation Rochester, MN.
Journal of Clinical Neuromuscular Disease
|December 17, 2008
Summary
Erythromelalgia involves painful, hot extremities. Studies reveal increased blood flow and temperature paradoxically decrease oxygenation, often linked to small and large fiber neuropathies.
Area of Science:
- Neurology
- Vascular Medicine
- Autonomic Neuroscience
Background:
- Erythromelalgia is a poorly understood condition causing painful, hot, red extremities.
- Understanding the underlying vascular, neurophysiologic, and autonomic dysfunction is crucial.
Purpose of the Study:
- To determine the frequency and types of abnormalities in vascular, peripheral neurophysiologic, and autonomic function in erythromelalgia patients.
- To elucidate the pathophysiology of erythromelalgia.
Main Methods:
- Analysis of 163 patient charts with a clinical diagnosis of erythromelalgia.
- Vascular studies (n=93), neurophysiologic testing (n=54), autonomic reflex screening (ARS, n=27), and peripheral autonomic surface potential (PASP) recordings (n=2).
Main Results:
- During symptoms, toe temperature increased by 11.6°C and laser flow increased significantly (P<.0.0001), while TcPO2 decreased (P=0.032).
- Abnormal electromyography (21/54) indicated axonal neuropathy. Severe postganglionic sudomotor impairment was found in 17/27 ARS and 1/2 PASP, with 5/17 also showing peripheral adrenergic dysfunction.
Conclusions:
- Erythromelalgia symptoms paradoxically involve increased flow/temperature with decreased oxygenation.
- A high proportion of patients exhibit distal small fiber neuropathy, particularly affecting cutaneous sympathetic fibers, often with coexisting large fiber neuropathy.
