Not to be confused with Harlequin type ichthyosis.
Medical condition
Harlequin syndrome
Other names
Progressive isolated segmental anhidrosis
A man exhibiting the asymmetric symptoms of Harlequin syndrome. One half of the forehead is more red than the other.
Harlequin syndrome is a condition characterized by asymmetric sweating and flushing on the upper thoracic region of the chest, neck and face. Harlequin syndrome is considered an injury to the autonomic nervous system (ANS). The ANS controls some of the body's natural processes such as sweating, skin flushing and pupil response to stimuli.[1] Individuals with this syndrome have an absence of sweat skin flushing unilaterally, usually on one side of the face, arms and chest. It is an autonomic disorder that may occur at any age.[2] Harlequin syndrome affects fewer than 1000 people in the United States.[3]
Symptoms associated with Harlequin syndrome are more likely to appear when a person has been in the following conditions: exercising, warm environment and intense emotional situation. Since one side of the body sweats and flushes appropriately to the condition, the other side of the body will have an absence of such symptoms.[4] This syndrome has also been called the "Harlequin sign" and thought to be one of the spectrum of diseases that may cause Harlequin syndrome.
It can also be the outcome of a one sided endoscopic thoracic sympathectomy (ETS) or endoscopic sympathetic blockade (ESB) surgery.[2][5]
Harlequin syndrome can also be seen as a complication of VA (veno-arterial) extracorporeal membrane oxygenation (ECMO). This involves differential hypoxemia (low oxygen levels in the blood) of the upper body in comparison to the lower body.[6]
^NIH - National Cancer Institute. "Autonomic Nervous System". PubMed Health.
^ abLance, J. W. (2005). "Harlequin syndrome". Practical Neurology. 5 (3): 176–177. doi:10.1111/j.1474-7766.2005.00306.x.
^"Different Sides of Harlequin Syndrome". Cleveland Clinic. Retrieved 2023-08-29.
^Cite error: The named reference :0 was invoked but never defined (see the help page).
^Wasner, G.; Maag, R.; Ludwig, J.; Binder, A.; Schattschneider, J.; Stingele, R.; Baron, R. (2005). "Harlequin syndrome - one face of many etiologies". Nature Clinical Practice Neurology. 1 (1): 54–59. doi:10.1038/ncpneuro0040. PMID 16932492. S2CID 5324849.
^Al Hanshi, Said Ali Masoud; Othmani, Farhana Al (2017). "A case study of Harlequin syndrome in VA-ECMO". Qatar Medical Journal. 2017 (1): 39. doi:10.5339/qmj.2017.swacelso.39. PMC 5474607.
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