Erythromelalgia as a form of neuropathy

Staub, David B.

doi:10.1001/archderm.128.12.1654

Cited by 12 publications

(12 citation statements)

References 4 publications

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“…The findings of reduced focal sympathetic activity are in keeping with histological evidence from three case reports, which showed a decrease in sympathetic innervation in EM patients [5,18,19]. Two of these patients had diabetes mellitus and reduced nerve conduction velocities [5,19].…”

Section: Discussionsupporting

confidence: 83%

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Impaired skin vasomotor reflexes in patients with erythromelalgia

Littleford¹,

Khan²,

Belch³

1999

Clinical Science

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Erythromelalgia (EM) is a chronic disorder characterized by intermittent burning pain, warmth and erythema of the extremities. Increasing the local temperature and dependency of the affected limb(s) precipitates the symptoms, whereas direct cooling and elevation of the limb(s) can provide partial relief. Our previous findings showed that patients with EM have enhanced cutaneous vascular tone at rest and during stimulation, which may be due to an increase in sympathetic neural activity. To test this, we measured skin vasoconstrictor responses to contralateral arm cold challenge (CC) and inspiratory gasp (IG) using laser Doppler flowmetry at the toe pulp and fingertip. These areas were chosen because of their dense sympathetic innervation. An index of the vasoconstrictor response (between 0 and 1) was calculated from the change in skin perfusion from baseline following CC and IG. In control subjects, vasoconstrictor responses to CC at the toe and fingertip were both 0. 70+/-0.02 (mean+/-S.E.M.), which were significantly greater (P<0. 001) than corresponding values in patients with EM (0.37+/-0.04 and 0.45+/-0.04 respectively). Similarly, vasoconstrictor responses to IG were significantly greater (P<0.001) at the toe and fingertip in control subjects (0.70+/-0.03 and 0.70+/-0.02 respectively) compared with values in EM patients (0.27+/-0.03 and 0.45+/-0.15 respectively). These data show that, in contrast with control subjects, patients with EM have diminished sympathetic vasoconstrictor responses to both CC and IG. Denervation supersensitivity may play a part by increasing vasoconstrictor responses to circulating catecholamines, leading to a reduction in skin blood flow. Therefore an interplay between neural and vasoactive agents may be involved in the pathophysiology of EM.

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Section: Discussionsupporting

confidence: 83%

“…Two of these patients had diabetes mellitus and reduced nerve conduction velocities [5,19]. A decreased number of acetylcholinesterase and catecholamine-containing nerve terminals in peri-arterial and sweat-gland plexi was observed in skin biopsies taken from the dorsum of the foot in these three EM patients.…”

Section: Discussionmentioning

confidence: 99%

Impaired skin vasomotor reflexes in patients with erythromelalgia

Littleford¹,

Khan²,

Belch³

1999

Clinical Science

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“…Cutaneous blood vessels are under sympathetic neural innervation [16], and thus a reduction in blood flow could result from increased sympathetic neural activity. However, histological evidence suggests that there is an actual reduction in sympathetic innervation [7,17,18]. A reduced number of acetylcholinesterase and catecholamine-containing nerve terminals in the peri-arterial and sweat gland plexuses has been observed in skin biopsies taken from the dorsum of the foot in three EM patients [7,17,18].…”

Section: Discussionmentioning

confidence: 99%

“…However, histological evidence suggests that there is an actual reduction in sympathetic innervation [7,17,18]. A reduced number of acetylcholinesterase and catecholamine-containing nerve terminals in the peri-arterial and sweat gland plexuses has been observed in skin biopsies taken from the dorsum of the foot in three EM patients [7,17,18]. Furthermore, our pilot work shows a decrease in sympathetically mediated vasoconstrictor ability in response to cold challenge and inspiratory gasp in EM patients (R. C. Littleford et al, unpublished observations).…”

Section: Discussionmentioning

confidence: 99%

“…They found abnormal skin temperature regulation after the induced nerve injury and showed that as a group the temperature of the rat's paw evolved from abnormally hot post-operatively to abnormally cold after 30 days. Individually however, the paw temperature could alternate from abnormally hot to abnormally cold and vice [7,17,18], Wakisaka and colleagues [19] found a decrease in the number of sympathetic effector nerves in the affected limb but normal sympathetic nerve innervation in the normal contralateral limb. Moreover, there was no typical sympathetic nerve pattern associated with the observed temperature change of the hind paws.…”

Section: Discussionmentioning

confidence: 99%

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Skin perfusion in patients with erythromelalgia

Littleford

Khan

Belch

1999

Eur J Clin Investigation

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Our study indicates for the first time that there is a vasoconstrictor tendency in patients with EM, which may be related to functional or structural changes in skin microvessels. Thus, the previous hypothesis that the pathophysiology of EM relates to vasodilatation is not supported in our patients. We believe that, in EM, vasoconstriction precedes reactive hyperaemia, similar to that seen in Raynaud's phenomenon.

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