[Erythromelalgia, arterial hypertension and increased excretion of urinary catecholamines]

A 7 year-old girl was hospitalized with acute, severe and drug resistant erythromelalgia. During her stay in the hospital, she presented with an epileptic seizure associated with hypertension (220-120 mmHg). Catecholamine urinary excretion was markedly increased. Diagnoses of pheochromocytoma and acrodynia were excluded. Erythromelalgia and hypertension both disappeared in a few days without any relapse after a 2 year-follow-up. Catecholamine urinary excretion returned to normal levels in a few weeks. A skin biopsy which was performed in an affected site, showed a slight and questionable reduction of the density of autonomic adrenergic nerve terminals in the periarterial and glandular plexuses. The relevance of transitory excess catecholamine excretion and its link with erythromelalgia and hypertension are discussed. The hypothesis that the disorder could be explained by an abnormality of distal autonomic axons is likely.