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Harlequin Syndrome
  1. James W. Lance, Honorary Consultant Neurologist and Emeritus Professor of Neurology
  1. Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, E-mail: jimlance{at}


Arleccino (Harlequin) was a character in the travelling improvizational theatre originating in Venice in the 16th century, known as Comedia Dell’ Arte. This remained popular until the 18th century, but many of the characters are still recognizable today – Columbine, Pagliacci and Pulcinella, a woman-chasing hunchback who was the forerunner of Punch. The first Harlequin masks were grotesque and the term Harlequin syndrome was therefore applied to a severe form of congenital ichthyosis with rudimentary ears, ectropion and ‘fish mouth’ appearance. But later, Harlequin masks became less sinister and more sophisticated with diamond-shaped colourful patterns, or blackening of one half of the face (Fig. 1). This appearance came to mind when my colleagues and I observed five patients who flushed and sweated excessively on one side of the face in hot weather, or after exercise, particularly obvious when the sweating area was demonstrated by the application of alizarin powder (Fig. 2)

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