Harlequin syndrome: two new cases and a management proposal

Acta Neurol Belg. 2009 Sep;109(3):214-20.


The Harlequin syndrome is a rare autonomic disorder, characterized by unilateral diminished sweating and flushing of the face in response to heat or exercise. We present two new cases and evaluate the data of 83 patients described in the literature. We provide diagnostic and therapeutic guidelines.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Autonomic Nervous System Diseases / etiology*
  • Autonomic Nervous System Diseases / physiopathology
  • Autonomic Nervous System Diseases / therapy*
  • Blushing
  • Female
  • Humans
  • Ichthyosis, Lamellar / complications*
  • Ichthyosis, Lamellar / physiopathology
  • Ichthyosis, Lamellar / therapy*
  • Male
  • Middle Aged
  • Physical Exertion
  • Practice Guidelines as Topic
  • Sweating