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MEDICINA E CHIRURGIA DELLA CAVIGLIA E DEL PIEDE
A Journal on Physiopathology and Surgery of the Foot
Indexed/Abstracted in: EMBASE, Scopus
Chirurgia Del Piede 2006 August;30(2):77-85
Erythromelalgia or Weir Mitchell’s syndrome. Past and present
Pisani G., Pisani P. C., Parino E.
Centro di Chirurgia del Piede “Prof. G. Pisani” Casa di Cura “Fornaca di Sessant”, Torino
Erythromelalgia is a rare disease which, while mainly involving the foot, is little known by those who work in foot pathology; on the other hand it is not uncommon to encounter it in neurological, dermatological, haematological, vascular, rheumatological and genetic bibliographies. Characterised by redness (erythros) of the extremity (melos) and in particular of the foot with pain (algos) very often starting at the big toe, it may occur in youthful or adult forms, the latter idiopathic or secondary. Pain episodes are exacerbated by heat and by the position of the extremity, while they are alleviated by raising the extremity and exposing it to the cold. Foot and tibial pulsations are marked. There are no evident associated pathologies in the youthful form and in the adult idiopathic form, while the secondary form, with a usually asymmetric localisation, is due to neurological or vascular pathologies and haemopathies, particularly poly and thrombocytaemia; in these cases aspirin is very helpful. Genetic changes have recently been hypothesised. While for secondary forms the aetiopathogenesis can be supposed, that of the youthful and adult idiopathic forms is unknown; in certain aspects however these forms are related to altered activity of the sympathetic nervous system with changes in blood circulation and in tissue trophism. Regional vasomotor disturbances might be due to an afferent sympathetic stimulus, part of a reflex diastaltic arc as hypothesised by Lerich for algodystrophy. Lesion of the sympathetic fibres? Lesion of the peripheral ganglia? Lesion of the cerebrospinal centres? The discussion is still open and can be continued on the basis of a revisiting of what was hypothesised by authors more than a century ago. Treatment is difficult. Over and above the treatment of the basic condition (haemopathy, neurological pathology, etc.), in the secondary forms, treatment is difficult particularly of the primary or essential forms. Exposure of the extremities to cold, and sedentary activity, is useful, Aspirin, pyroxan, indomethacin may temporarily attenuate the pain episodes. Beta blockers may be useful as a maintenance therapy. Neurectomy, sympatheticectomy are also proposed.