Harlequin Sign

Harlequin Sign

The harlequin sign is asymmetrical facial flushing with sweating after exercise. That it reflects localized autonomic dysfunction may be indicated by its associations with congenital Horner’s syndrome, and as one element in the spectrum of Holmes-Adie syndrome and Ross’s syndrome. Harlequin sign has on occasion been described in association with multiple sclerosis and superior mediastinal neurinoma.


Carroll CB, Zajicek JP. The "harlequin" sign in association with multiple sclerosis. Journal of Neurology 2004; 251: 1145-1146
Lance JW, Drummond PD, Gandevia SC, Morris JGL. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. Journal of Neurology, Neurosurgery and Psychiatry 1988; 51: 635-642


Cross References

Holmes-adie pupil, Holmes-adie syndrome; Horner’s syndrome