MCID: HRL002
MIFTS: 25

Harlequin Syndrome

Categories: Neuronal diseases, Rare diseases, Skin diseases

Aliases & Classifications for Harlequin Syndrome

MalaCards integrated aliases for Harlequin Syndrome:

Name: Harlequin Syndrome 53 59 29 72
Progressive Isolated Segmental Anhidrosis 53 59
Unilateral Loss of Facial Flushing and Sweating with Contralateral Anhidrosis 53
Sudden Onset of Unilateral Flushing and Sweating 53

Characteristics:

Orphanet epidemiological data:

59
harlequin syndrome
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

MESH via Orphanet 45 C535634
ICD10 via Orphanet 34 G90.8
UMLS via Orphanet 73 C2029348
Orphanet 59 ORPHA199282
UMLS 72 C2029348

Summaries for Harlequin Syndrome

NIH Rare Diseases : 53 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 : 75 Harlequin syndrome is a condition characterized by asymmetric sweating and flushing on the upper... more...

Related Diseases for Harlequin Syndrome

Diseases related to Harlequin Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 34, show less)
# Related Disease Score Top Affiliating Genes
1 horner's syndrome 10.5
2 anhidrosis 10.3
3 adie pupil 10.2
4 headache 10.2
5 ptosis 10.1
6 goiter 10.1
7 migraine with or without aura 1 10.0
8 neuroblastoma 1 10.0
9 autonomic neuropathy 10.0
10 neurilemmoma 10.0
11 cluster headache 10.0
12 autonomic dysfunction 10.0
13 anisocoria 9.9
14 horner syndrome, congenital 9.9
15 hyperhidrosis, gustatory 9.9
16 multiple system atrophy 1 9.9
17 chondrosarcoma 9.9
18 carney triad 9.9
19 pain agnosia 9.9
20 guillain-barre syndrome 9.9
21 brain cancer 9.9
22 retinal vein occlusion 9.9
23 constipation 9.9
24 gangliocytoma 9.9
25 papillary carcinoma 9.9
26 ganglioneuroma 9.9
27 hyperthyroidism 9.9
28 neuropathy 9.9
29 autoimmune autonomic ganglionopathy 9.9
30 primary orthostatic hypotension 9.9
31 pure autonomic failure 9.9
32 dysautonomia 9.9
33 paresthesia 9.9
34 posttransplant acute limbic encephalitis 9.9

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:

41
Skin, Testes, Lung, Pituitary

Publications for Harlequin Syndrome

Articles related to Harlequin Syndrome:

(showing 105, show less)
# Title Authors PMID Year
1
Pure autonomic failure presenting as Harlequin syndrome. 38
31331696 2019
2
Harlequin Syndrome in Acute Thalamic Hemorrhage. 38
31301985 2019
3
Harlequin Syndrome Associated with Erector Spinae Plane Block. 38
31021847 2019
4
[Harlequin syndrome secondary to a neuroblastoma]. 38
30007875 2019
5
Branch Retinal Vein Occlusion in Ipsilateral Harlequin Syndrome. 38
30999345 2019
6
Cross-Field Ventilation for Treatment of Harlequin Syndrome in Traumatic Tracheobronchial Injury Repair Using Intraoperative Venoarterial Extracorporeal Membrane Oxygenation. 38
31076308 2019
7
Harlequin syndrome in a paediatric patient: A diagnostic challenge. 38
27296496 2018
8
[Harlequin syndrome: Two sides of the same disease]. 38
29599062 2018
9
Child Neurology: An infant with episodic facial flushing: A rare case and review of congenital harlequin syndrome. 38
30082439 2018
10
Harlequin Syndrome. 38
29238939 2018
11
Do you know this syndrome? Harlequin syndrome. 38
30066772 2018
12
Position of draining venous cannula in extracorporeal membrane oxygenation for respiratory and respiratory/circulatory support in adult patients. 38
29907121 2018
13
Harlequin syndrome as a rare complication after epidural anesthesia in an obstetric patient. 38
29343042 2018
14
Postoperative Harlequin Syndrome: Case Report of a Rare but Clinically Striking Condition. 38
29652689 2018
15
Harlequin syndrome following upper lobectomy. 38
29411633 2018
16
Controlled flow diversion in hybrid venoarterial-venous extracorporeal membrane oxygenation. 38
29155934 2018
17
Unilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome. 38
28799533 2017
18
Harlequin syndrome and Horner syndrome after neck schwannoma excision in a pediatric patient: A case report. 38
29137067 2017
19
Three Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula. 38
28689949 2017
20
Development of Harlequin Syndrome following placement of thoracic epidural anesthesia in a pediatric patient undergoing Nuss procedure. 38
28878918 2017
21
Harlequin sign concomitant with Horner syndrome after anterior cervical discectomy: a case of intrusion into the cervical sympathetic system. 38
28291413 2017
22
Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma. 38
28382275 2017
23
Idiopathic Harlequin Syndrome Manifesting during Exercise: A Case Report and Review of the Literature. 38
28316628 2017
24
A pediatric case of idiopathic Harlequin syndrome. 38
28018464 2016
25
Harlequin Syndrome Associated with Multilevel Intercostal Nerve Block. 38
27306195 2016
26
Harlequin Syndrome after Stellate Ganglia Neuroblastoma Resection. 38
27344219 2016
27
Harlequin syndrome with contralateral anhidrosis after an upper chest gunshot wound. 38
27535737 2016
28
[Harlequin syndrome in a melanoderm patient]. 38
26964837 2016
29
Harlequin Syndrome in Cluster Headache. 38
27238298 2016
30
[Harlequin syndrome]. 38
27188995 2016
31
Congenital Harlequin syndrome as an isolated phenomenon: A case report and review of the literature. 38
26943985 2016
32
Harlequin Syndrome After Thoracic Paravertebral Block. 38
26513674 2016
33
Botulinum toxin for treatment of Harlequin syndrome. 38
26750113 2016
34
A Case of Undiagnosed Harlequin Syndrome Presenting in General Dental Practice. 38
27024906 2016
35
Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. 38
26704065 2016
36
Harlequin Syndrome Following Implantation of Intrathecal Pumps: A Case Series. 38
26399375 2015
37
Harlequin Syndrome Associated with Thoracic Epidural Analgesia. 38
26039330 2015
38
Hemifacial Erythema in Right Unilateral Electroconvulsive Therapy. 38
25634567 2015
39
Concurrent Horner's and Harlequin syndromes. 38
25662430 2015
40
Harlequin syndrome: a mask of rare dysautonomic syndromes. 38
25933078 2015
41
Harlequin syndrome, a rare neurological disease. 38
23810290 2015
42
Iatrogenic harlequin syndrome: a new case. 38
25673945 2015
43
[A woman with unilateral flushing of her face]. 38
25923501 2015
44
Pitfalls in percutaneous ECMO cannulation. 38
26811838 2015
45
Harlequin syndrome as a complication of epidural anesthesia. 38
24882829 2014
46
Harlequin syndrome after thyroidectomy for compressive retrosternal goiter. Case report and review of the literature. 38
25102714 2014
47
[Evaluation of sudomotor function]. 38
25672702 2014
48
Apical schwannoma presenting as harlequin syndrome. 38
24296202 2013
49
Autonomic dysfunction manifesting with asymmetric face flushing and paroxysmal nonconvulsive episodes. 38
23112241 2013
50
[Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature]. 38
23726525 2013
51
Novel management of harlequin syndrome with stellate ganglion block. 38
23909827 2013
52
Postexertional harlequin syndrome with spontaneous improvement. 38
24000217 2013
53
Idiopathic harlequin syndrome: a pediatric case. 38
22638078 2013
54
Pulmonary alveolar proteinosis requiring "hybrid" extracorporeal life support, and complicated by acute necrotizing pneumonia. 38
23523160 2013
55
Harlequin syndrome in childhood--case report. 38
23197213 2012
56
Harlequin syndrome after extrapleural bupivacaine infusion. 38
22782989 2012
57
First report of harlequin syndrome as the presenting feature of Carney Triad: a diagnostic and imaging challenge. 38
22689797 2012
58
Headache in three new cases of Harlequin syndrome with accompanying pharmacological comparison with migraine. 38
22492215 2012
59
Harlequin syndrome: does a cranial autonomic neuropathy influence headache? 38
22523398 2012
60
Harlequin syndrome post-transsphenoidal pituitary macroadenoma surgery. 38
22801568 2012
61
Treatment of Harlequin syndrome. 38
22072130 2012
62
An old lady with anterior chest pain and unilateral facial flushing. 38
20971594 2012
63
Harlequin syndrome after jogging. 38
21895600 2011
64
[Harlequin syndrome]. 38
20303123 2011
65
Treatment of Harlequin syndrome by costotransversectomy and sympathectomy: case report. 38
21796071 2011
66
Unilateral facial flushing and sweating after physical exercise: Harlequin syndrome. 38
21271501 2011
67
Harlequin syndrome caused by lesion of sympathetic regulatory neurons. 38
21321344 2011
68
Harlequin syndrome in a case of toxic goitre: a rare association. 38
21785603 2011
69
A case of idiopathic harlequin syndrome. 38
22041394 2011
70
Teaching neuroimages: harlequin syndrome caused by lesion of sympathetic regulatory neurons. 38
20548037 2010
71
Harlequin syndrome: two new cases and a management proposal. 38
19902816 2009
72
Harlequin syndrome. 38
19559258 2009
73
Coexisting harlequin and Horner syndromes after paediatric neck dissection: a case report and a review of the literature. 38
17556055 2008
74
Harlequin syndrome in spontaneous dissection of the cervical carotid artery. 38
18955692 2008
75
Pupillographic findings in 39 consecutive cases of harlequin syndrome. 38
18769278 2008
76
Harlequin syndrome: still only half understood. 38
18769277 2008
77
[Harlequin syndrome secondary to thoracic chondrosarcoma]. 38
18327749 2008
78
Unnoticed dysautonomic syndrome of the face: Harlequin syndrome. 38
17569597 2007
79
Transient postoperative harlequin syndrome combined with Horner's syndrome in a pediatric patient after neck mass excision. 38
17498026 2007
80
Intraoperative Harlequin syndrome. 38
17312248 2007
81
Intraoperative Harlequin syndrome. 38
16428595 2006
82
Harlequin syndrome--one face of many etiologies. 38
16932492 2005
83
Harlequin syndrome with crossed sympathetic deficit of the face and arm. 38
15832011 2005
84
Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy. 38
15756124 2005
85
Harlequin syndrome in two athletes. 38
15618325 2005
86
Harlequin syndrome: an association with overlap parasomnia. 38
14742626 2004
87
Segmental anhidrosis with hyporeflexia associated with congenital spinal deformity: a Ross's syndrome variant or inverse Horner's syndrome? 38
17642554 2004
88
Iatrogenic harlequin syndrome. 38
12782774 2003
89
Harlequin syndrome following internal jugular vein catheterization in an adult under general anesthetic. 38
12357195 2002
90
Paroxysmal episodic central thermoregulatory failure. 38
11971107 2002
91
Ocular management of harlequin syndrome. 38
11231785 2001
92
[Harlequin syndrome]. 38
11057192 2000
93
Harlequin syndrome. 38
10201435 1999
94
Skin conductance responses in paediatric Harlequin syndrome. 38
10189659 1999
95
[Harlequin syndrome]. 38
9555141 1997
96
Postoperative autonomic deficit: a case of harlequin syndrome. 38
8916840 1996
97
Sweating and vascular responses in the face: normal regulation and dysfunction in migraine, cluster headache and harlequin syndrome. 38
7888747 1994
98
Site of autonomic deficit in harlequin syndrome: local autonomic failure affecting the arm and the face. 38
8250530 1993
99
Unilateral loss of facial flushing and sweating with contralateral anhidrosis: harlequin syndrome or Adie's syndrome? 38
8292878 1993
100
Harlequin syndrome due to superior mediastinal neurinoma. 38
1809267 1991
101
Nursing a special patient: the infant with congenital icthyosis--harlequin syndrome. 38
2038278 1991
102
[Harlequin syndrome (unilateral flushing and sweating attack) due to a spinal invasion of the left apical lung cancer]. 38
2331828 1990
103
Harlequin syndrome: the sudden onset of unilateral flushing and sweating. 38
3155385 1988
104
[Prenatal diagnosis of a harlequin fetus using electron microscopy]. 38
6667288 1983
105
Progressive isolated segmental anhidrosis. 38
7065936 1982

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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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