034. Channelling pain - Reducing anxiety reduces pain in a particularly severe case primary erythromelalgia

Abstract

Background: A four year old girl presents with red painful swollen feet. Pain is worse when warm or anxious. A genetic mutation is identified in the SCN9A sodium channel gene and the diagnosis is primary erythromelalgia, This mutation is associated with large electrophysiological changes and having been identified once previously in a child in the United States of America is the only known case in the UK with this mutation. Aim(s): We describe two complications of the condition firstly a cold thermal injury and second large skin ulcers. This case also demonstrates that reducing anxiety alongside careful skin management can dramatically reduce medication usage and improve quality of life in this case. Mexiletine, a non-selective sodium channel blocker medication, may demonstrate some benefit. Method(s): With less than 10 known cases of paediatric primary erythromelalgia in the UK we discuss the important findings from this particularly severe case. Sodium channels are used in the sympathetic ganglia and peripheral nerve fibres demonstrating a clear physiological possible reason why reducing anxiety reduces the effects of this condition in this case. Result(s): This case also demonstrates the intermittent pyrexias and hypertensive episodes that can be associated with primary erythromelalgia. Conclusion(s): It is important to collaborate with other centres nationally with the aim to benefit the children affected by primary erythromelalgia. DA