MCID: HRL002
MIFTS: 23

Harlequin Syndrome

Categories: Neuronal diseases, Rare diseases, Skin diseases

Aliases & Classifications for Harlequin Syndrome

MalaCards integrated aliases for Harlequin Syndrome:

Name: Harlequin Syndrome 52 58 29 71
Progressive Isolated Segmental Anhidrosis 52 58
Unilateral Loss of Facial Flushing and Sweating with Contralateral Anhidrosis 52
Sudden Onset of Unilateral Flushing and Sweating 52

Characteristics:

Orphanet epidemiological data:

58
harlequin syndrome
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

MESH via Orphanet 44 C535634
ICD10 via Orphanet 33 G90.8
UMLS via Orphanet 72 C2029348
Orphanet 58 ORPHA199282
UMLS 71 C2029348

Summaries for Harlequin Syndrome

NIH Rare Diseases : 52 Harlequin syndrome is a syndrome affecting the autonomic nervous system . The autonomic nervous system is responsible for controlling the body's natural processes such as sweating , skin flushing , and the response of the pupils to stimuli. People with Harlequin syndrome have the absence of sweating and flushing of skin on one side of the body (unilateral), especially of the face, arms, and chest. The symptoms associated with Harlequin syndrome may be more likely to occur when a person has been exercising, is very warm, or is in an intense emotional situation. In these situations, one side of the body sweats and flushes appropriately as a response to the situation, whereas the other side of the body does not. The asymmetrical facial sweating and flushing associated with this condition has been named the "Harlequin sign." Harlequin syndrome is thought to be one of a spectrum of diseases that can cause Harlequin sign. The exact cause of Harlequin syndrome is not completely understood. In some patients with this syndrome, the underlying cause seems to be a lesion or tumor that is affecting the ability of the cells of the autonomic nervous system to communicate with one side of the body. However, in many cases an exact cause of the symptoms is not found. Diagnosis of Harlequin syndrome is based on observing symptoms consistent with the syndrome, followed by a series of tests to rule out other diseases associated with Harlequin sign. Treatment may consist of removing any lesion that may be causing the symptoms of the syndrome. If no lesion is present and the syndrome is not interfering with a person's daily living, treatment may not be necessary.

MalaCards based summary : Harlequin Syndrome, also known as progressive isolated segmental anhidrosis, is related to horner's syndrome and anhidrosis, and has symptoms including flushing Affiliated tissues include skin, testes and lung.

Wikipedia : 74 Harlequin syndrome is a condition characterized by asymmetric sweating and flushing on the upper... more...

Related Diseases for Harlequin Syndrome

Graphical network of the top 20 diseases related to Harlequin Syndrome:



Diseases related to Harlequin Syndrome

Symptoms & Phenotypes for Harlequin Syndrome

UMLS symptoms related to Harlequin Syndrome:


flushing

Drugs & Therapeutics for Harlequin Syndrome

Search Clinical Trials , NIH Clinical Center for Harlequin Syndrome

Genetic Tests for Harlequin Syndrome

Genetic tests related to Harlequin Syndrome:

# Genetic test Affiliating Genes
1 Harlequin Syndrome 29

Anatomical Context for Harlequin Syndrome

MalaCards organs/tissues related to Harlequin Syndrome:

40
Skin, Testes, Lung, Pituitary, Brain

Publications for Harlequin Syndrome

Articles related to Harlequin Syndrome:

(show top 50) (show all 111)
# Title Authors PMID Year
1
Harlequin syndrome caused by isolated focal parasympathetic neuropathy. 61
31768686 2020
2
North-south syndrome in veno-arterial extra-corporeal membrane oxygenator: the other Harlequin syndrome. 61
31598907 2020
3
[Harlequin syndrome after scoliosis surgery]. 61
31620827 2019
4
Cross-Field Ventilation for Treatment of Harlequin Syndrome in Traumatic Tracheobronchial Injury Repair Using Intraoperative Venoarterial Extracorporeal Membrane Oxygenation. 61
31076308 2019
5
Harlequin Syndrome Associated with Erector Spinae Plane Block. 61
31021847 2019
6
Pure autonomic failure presenting as Harlequin syndrome. 61
31331696 2019
7
Harlequin Syndrome in Acute Thalamic Hemorrhage. 61
31301985 2019
8
[Harlequin syndrome secondary to a neuroblastoma]. 61
30007875 2019
9
Branch Retinal Vein Occlusion in Ipsilateral Harlequin Syndrome. 61
30999345 2019
10
Harlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF). 61
31579642 2019
11
Idiopathic harlequin syndrome: a case report and literature review. 61
31558939 2019
12
[Harlequin syndrome: Two sides of the same disease]. 61
29599062 2018
13
Harlequin syndrome in a paediatric patient: A diagnostic challenge. 61
27296496 2018
14
Child Neurology: An infant with episodic facial flushing: A rare case and review of congenital harlequin syndrome. 61
30082439 2018
15
Harlequin Syndrome. 61
29238939 2018
16
Do you know this syndrome? Harlequin syndrome. 61
30066772 2018
17
Position of draining venous cannula in extracorporeal membrane oxygenation for respiratory and respiratory/circulatory support in adult patients. 61
29907121 2018
18
Postoperative Harlequin Syndrome: Case Report of a Rare but Clinically Striking Condition. 61
29652689 2018
19
Harlequin syndrome as a rare complication after epidural anesthesia in an obstetric patient. 61
29343042 2018
20
Harlequin syndrome following upper lobectomy. 61
29411633 2018
21
Isolated Harlequin Syndrome Following Brachial Plexus Nerve Block via Interscalene Approach: A Case Report. 61
31573494 2018
22
Controlled flow diversion in hybrid venoarterial-venous extracorporeal membrane oxygenation. 61
29155934 2018
23
Unilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome. 61
28799533 2017
24
Harlequin syndrome and Horner syndrome after neck schwannoma excision in a pediatric patient: A case report. 61
29137067 2017
25
Three Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula. 61
28689949 2017
26
Development of Harlequin Syndrome following placement of thoracic epidural anesthesia in a pediatric patient undergoing Nuss procedure. 61
28878918 2017
27
Harlequin sign concomitant with Horner syndrome after anterior cervical discectomy: a case of intrusion into the cervical sympathetic system. 61
28291413 2017
28
Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma. 61
28382275 2017
29
Idiopathic Harlequin Syndrome Manifesting during Exercise: A Case Report and Review of the Literature. 61
28316628 2017
30
A pediatric case of idiopathic Harlequin syndrome. 61
28018464 2016
31
Harlequin Syndrome Associated with Multilevel Intercostal Nerve Block. 61
27306195 2016
32
Harlequin Syndrome after Stellate Ganglia Neuroblastoma Resection. 61
27344219 2016
33
Harlequin syndrome with contralateral anhidrosis after an upper chest gunshot wound. 61
27535737 2016
34
Harlequin Syndrome in Cluster Headache. 61
27238298 2016
35
[Harlequin syndrome in a melanoderm patient]. 61
26964837 2016
36
Congenital Harlequin syndrome as an isolated phenomenon: A case report and review of the literature. 61
26943985 2016
37
[Harlequin syndrome]. 61
27188995 2016
38
Harlequin Syndrome After Thoracic Paravertebral Block. 61
26513674 2016
39
Botulinum toxin for treatment of Harlequin syndrome. 61
26750113 2016
40
A Case of Undiagnosed Harlequin Syndrome Presenting in General Dental Practice. 61
27024906 2016
41
Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. 61
26704065 2016
42
Harlequin Syndrome Following Implantation of Intrathecal Pumps: A Case Series. 61
26399375 2015
43
Harlequin Syndrome Associated with Thoracic Epidural Analgesia. 61
26039330 2015
44
Hemifacial Erythema in Right Unilateral Electroconvulsive Therapy. 61
25634567 2015
45
Concurrent Horner's and Harlequin syndromes. 61
25662430 2015
46
Harlequin syndrome, a rare neurological disease. 61
23810290 2015
47
Harlequin syndrome: a mask of rare dysautonomic syndromes. 61
25933078 2015
48
Iatrogenic harlequin syndrome: a new case. 61
25673945 2015
49
[A woman with unilateral flushing of her face]. 61
25923501 2015
50
Pitfalls in percutaneous ECMO cannulation. 61
26811838 2015

Variations for Harlequin Syndrome

Expression for Harlequin Syndrome

Search GEO for disease gene expression data for Harlequin Syndrome.

Pathways for Harlequin Syndrome

GO Terms for Harlequin Syndrome

Sources for Harlequin Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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