Harlequin Syndrome

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Classification according to ICD-10
G90.8 Other diseases of the autonomic nervous system
ICD-10 online (WHO version 2019)

The Harlequin syndrome is a disorder of the autonomic nervous system with unilateral erythema and sweating usually only in the face , sometimes on the arm or chest . The disease can occur at any age, often in a warm environment or under stress.

The syndrome can be viewed as a special form of focal hyperhidrosis .

Synonyms are: progressive isolated segmental anhidrosis

The first description is from 1988 by JW Lance and colleagues.

However, NB Esterly and MK Spraker reported this phenomenon in newborns as early as 1985.

The disease should not be confused with harlequin ichthyosis .

distribution

The frequency is given as less than 1 in 1,000,000.

root cause

It is based either on damage to the sympathetic nerve in its course (peripheral), for example as a result of an endoscopic transthoracic sympathectomy or blockade or further thoracic operations, also as a complication after ECMO , or centrally in the brain stem .

The phenomenon is said to also occur idiopathically (with no apparent damage).

Clinical manifestations

Clinical criteria are:

literature

  • A. Lefevre, G. Schnepper: Development of Harlequin Syndrome following placement of thoracic epidural anesthesia in a pediatric patient undergoing Nuss procedure. In: Clinical case reports. Vol. 5, No. 9, September 2017, pp. 1523-1525, doi: 10.1002 / ccr3.1097 , PMID 28878918 , PMC 5582228 (free full text).
  • YJ Jeon, J. Son, JH Cho: Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma. In: The Korean journal of thoracic and cardiovascular surgery. Vol. 50, No. 2, April 2017, pp. 130-132, doi: 10.5090 / kjtcs.2017.50.2.130 , PMID 28382275 , PMC 5380209 (free full text).

Individual evidence

  1. ↑ a b c Harlequin syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ a b c T. Schlereth, M. Dieterich, F. Birklein: Hyperhidrosis - causes and therapy of excessive sweating. In: Deutsches Arzteblatt international. Vol. 106, No. 3, January 2009, pp. 32-37, doi: 10.3238 / arztebl.2009.0032 , PMID 19564960 , PMC 2695293 (free full text) (review).
  3. ↑ JW Lance, PD Drummond, SC Gandevia, JG Morris: Harlequin syndrome: the sudden onset of unilateral flushing and sweating. In: Journal of Neurology, Neurosurgery, and Psychiatry . Vol. 51, No. 5, May 1988, pp. 635-642, PMID 3155385 , PMC 1033068 (free full text).
  4. ↑ NB Esterly, MK Spraker: Neonatal skin problems. In: SL Moschella (ed.): Dermatology. Vol. 2nd edition, 1985, p. 1882.
  5. ↑ G. Wasner, R. Maag, J. Ludwig, A. Binder, J. Schattschneider, R. Stingele, R. Baron: Harlequin syndrome - one face of many etiologies. In: Nature clinical practice. Neurology. Vol. 1, No. 1, November 2005, pp. 54-59, doi: 10.1038 / ncpneuro0040 , PMID 16932492 .
  6. ^ L. Rupprecht, D. Lunz, A. Philipp, M. Lubnow, C. Schmid: Pitfalls in percutaneous ECMO cannulation. In: Heart, lung and vessels. Vol. 7, No. 4, 2015, pp. 320-326, PMID 26811838 , PMC 4712035 (free full text).
  7. ↑ CZ Yu Phuan, HL Tey: Unilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome. In: Indian journal of dermatology, venereology and leprology. [electronic publication before going to press] August 2017, PMID 28799533 .
  8. ↑ H. Algahtani, B. Shirah, R. Algahtani, A. Alkahtani: Idiopathic Harlequin Syndrome Manifesting during Exercise: A Case Report and Review of the Literature. In: Case reports in medicine. Vol. 2017, 2017, p. 5342593, doi: 10.1155 / 2017/5342593 , PMID 28316628 , PMC 5339527 (free full text).

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