Harlequin syndrome
| 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, also known as "harlequin sign", 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]
Symptoms associated with Harlequin syndrome are more likely to appear under the following conditions: vigorous exercise, warm environments and intense emotional situations. 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.[3]
Harlequin syndrome can alternatively be the outcome of a one-sided endoscopic thoracic sympathectomy (ETS) or endoscopic sympathetic blockade (ESB) surgery.[2][4] It can also be observed as a complication of 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.[5]
- ^ NIH - National Cancer Institute. "Autonomic Nervous System". PubMed Health.
- ^ a b Lance, J. W. (2005). "Harlequin syndrome". Practical Neurology. 5 (3): 176–177. doi:10.1111/j.1474-7766.2005.00306.x.
- ^ Cite error: The named reference
:0was 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.