A Síndrome de Harlequin (HSD) é uma condição que afeta o sistema nervoso autônomo (aquele que controla funções automáticas do corpo) e pode surgir em qualquer idade. Ela se caracteriza por vermelhidão e suor em apenas um lado do rosto (e, às vezes, do braço e do peito), que são desencadeados por calor, exercícios físicos ou estresse emocional. No entanto, não há sintomas nos olhos relacionados à parte simpática do sistema nervoso. Porém, em casos raros, a síndrome pode causar pupilas que reagem de forma anormal, uma lesão nos nervos que controlam os movimentos dos olhos e as pupilas (ligados ao sistema parassimpático) e uma falha do sistema nervoso simpático na capacidade de produzir suor.
Introdução
O que você precisa saber de cara
A Síndrome de Harlequin (HSD) é uma condição que afeta o sistema nervoso autônomo (aquele que controla funções automáticas do corpo) e pode surgir em qualquer idade. Ela se caracteriza por vermelhidão e suor em apenas um lado do rosto (e, às vezes, do braço e do peito), que são desencadeados por calor, exercícios físicos ou estresse emocional. No entanto, não há sintomas nos olhos relacionados à parte simpática do sistema nervoso. Porém, em casos raros, a síndrome pode causar pupilas que reagem de forma anormal, uma lesão nos nervos que controlam os movimentos dos olhos e as pupilas (ligados ao sistema parassimpático) e uma falha do sistema nervoso simpático na capacidade de produzir suor.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 10 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome de Harlequin
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Publicações mais relevantes
Laser therapy: palliative care for the Harlequin syndrome?
Harlequin syndrome is a rare condition characterized by facial dysautonomia with anhidrosis, hemifacial blush, and other nonfacial manifestations without conventional treatment. This report presents a case study of the potential treatment of Harlequin syndrome using laser therapy. A single-case AB design was used, allowing for a systematic comparison between the baseline and intervention phases. The baseline phase involved data collection during a period without intervention (control condition), that is before laser therap. As evaluation criteria, a clinical assessment of skin turgor, sweating or anhidrosis, and blushing was conducted. Additionally, photographic records were taken before and after treatment, and the patient satisfaction index was analyzed. The Treatment Satisfaction Questionnaire for Medication was used as a model, with adaptations made for specific patient cases. The intervention phase included laser therapy. The patient was an 18-year old female diagnosed with Harlequin syndrome without associated comorbidities. The patient was instructed not to undergo any treatment other than the laser therapy. After the laser therapy, the patient reported improvement in symptoms and satisfaction with the outcome and the therapy. It was concluded that, despite being a rare syndrome with limited available information, laser therapy is a viable and efficient approach for treating Harlequin syndrome.
A 17-year-old boy with hemifacial flushing and anhidrosis.
Case report of a cardiac Harlequin syndrome-electrical storm during venoarterial extracorporeal membrane oxygenation.
In sepsis-induced cardiogenic shock, venoarterial extracorporeal membrane oxygenation (VA-ECMO) can improve survival. Simultaneous acute respiratory distress syndrome (ARDS) increases the risk of differential hypoxia (Harlequin syndrome). Due to desaturated blood ejected by the heart, the head becomes blue, whereas the lower body remains oxygenated by VA-ECMO. We report on an unusual cardiac manifestation, leading to electrical storm. We present the clinical case of a 55-year-old man. During a minor viral pneumonia, superinfection led to severe ARDS and sepsis-induced refractory cardiogenic shock. Venoarterial extracorporeal membrane oxygenation support was initiated. In progressive respiratory failure, an electrocardiogram (ECG) revealed the onset of ST-segment elevations mirroring hypoxic coronary perfusion. As the mixing zone of blood from the heart and the VA-ECMO was in the ascending aorta, hypoxia was limited to the heart. Ventricular arrhythmias recurred, until ventricular fibrillation remained refractory to defibrillation. A second return cannula was inserted into the jugular vein, and veno-arteriovenous ECMO (V-AV-ECMO) was established. After the venous return was added to the circuit, ventricular fibrillation was defibrillated and sinus rhythm remained stable. Within an hour, ST-elevations receded. Systolic function recovered to normal within 26 days. In severe sepsis-related cardiogenic shock, cardiac output is likely to recover. Venoarterial extracorporeal membrane oxygenation is a potential bridge to recovery. Apart from textbook knowledge, Harlequin syndrome can exclusively cause coronary ischaemia, leading to ST-segment elevations and electrical storm. ECGs reveal ST-elevations for early detection. Isolated cardiac Harlequin syndrome can be overlooked or misinterpreted as result of coronary artery disease, but needs immediate therapy to save the patient's life (e.g. V-AV-ECMO).
Harlequin syndrome as part of cervicothoracic spine injury following a fall.
Routine Screening for Neuroblastoma in Harlequin Syndrome Without Otherwise Known Etiology.
Publicações recentes
Harlequin Syndrome with Ipsilateral Facial Flushing After Internal Carotid Artery Dissection: An Atypical Sympathetic Laterality Pattern.
Harlequin syndrome: segmental autonomic dysfunction in the realm of Sjogren's disease.
A 17-year-old boy with hemifacial flushing and anhidrosis.
A pediatric case of idiopathic harlequin syndrome.
Laser therapy: palliative care for the Harlequin syndrome?
📚 EuropePMC163 artigos no totalmostrando 138
A 17-year-old boy with hemifacial flushing and anhidrosis.
JAAD case reportsA pediatric case of idiopathic harlequin syndrome.
Dermatology online journalLaser therapy: palliative care for the Harlequin syndrome?
Einstein (Sao Paulo, Brazil)Post-marketing safety assessment of epinephrine: an analysis of the US FDA adverse event reporting system.
Frontiers in medicineIdiopathic Harlequin Syndrome: A Case Report of an Uncommon Disease.
CureusHarlequin syndrome following paravertebral block adjacent to a stretched pleura: a case report.
BMC anesthesiologyLeft robotic assisted thoracic surgery (RATS) stellate ganglion ganglioneuroma resection with post-operative Harlequin syndrome.
Multimedia manual of cardiothoracic surgery : MMCTSHarlequin Syndrome in Venoarterial ECMO and ECPELLA: When ECMO and Native or Impella Circulations Collide - A Comprehensive Review.
Reviews in cardiovascular medicineFacial Flushing (Harlequin Syndrome) Following Radiofrequency Ablation Of The Trigeminal Ganglion: A Case Report.
Pain medicine case reportsUnmasking the Harlequin syndrome: A clinical transformation.
JAAD case reportsHarlequin syndrome as part of cervicothoracic spine injury following a fall.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyHarlequin syndrome: using clinical features and autonomic testing to unmask the disorder.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyHarlequin Syndrome in a Post-COVID-19 Infection Elderly: A Case Report.
Acta neurologica TaiwanicaHarlequin Syndrome Caused by Multinodular Goitre.
CureusDifferential Venous Drainage Mimicking Differential Oxygenation in Patients on Extracorporeal Life Support.
ASAIO journal (American Society for Artificial Internal Organs : 1992)Delayed Presentation of Horner's and Harlequin-Like Symptoms Following Interscalene Peripheral Nerve Catheter Placement.
CureusHarlequin syndrome in a young patient with osteosarcoma after pleural metastasectomy: A case report and a mini review of the literature.
Experimental and therapeutic medicineCase report of a cardiac Harlequin syndrome-electrical storm during venoarterial extracorporeal membrane oxygenation.
European heart journal. Case reportsRoutine Screening for Neuroblastoma in Harlequin Syndrome Without Otherwise Known Etiology.
Pediatric blood & cancerAlgorithm of High-Risk Massive Pulmonary Thromboembolism with Extracorporeal Membrane Oxygenation.
Journal of clinical medicineCongenital Insensitivity to Pain With Anhidrosis Is Associated With Harlequin Color Change: A Survey Study.
Pediatric dermatologyImages in Vascular Medicine: Analysis of skin microvascular dysfunction in Harlequin syndrome using laser speckle contrast imaging.
Vascular medicine (London, England)Harlequin sign due to an upper thoracic paravertebral lesion. A systematic review of the literature.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaHarlequin Syndrome: A Rare Association With Migraine.
NeurologyHarlequin syndrome in a patient with probable hemicrania continua and exertional headache - is there a link? a case report.
BMC neurologyWhat can a thermal imaging camera tell you? Idiopathic Harlequin Syndrome.
Neurologia i neurochirurgia polskaIdiopathic Harlequin Syndrome.
Neurology IndiaRecognition of a patient with neck autonomic dysfunction: findings from a rare case report of harlequin syndrome in direct access physiotherapy.
The Journal of manual & manipulative therapyBlood flow and emboli transport patterns during venoarterial extracorporeal membrane oxygenation: A computational fluid dynamics study.
Computers in biology and medicineHarlequin syndrome: a picture speaks a thousand words.
Medicina intensivaBrain death determination in patients with veno-arterial extracorporeal membrane oxygenation: A systematic study to address the Harlequin syndrome.
Journal of critical careTo identify Harlequin syndrome in patients with venoarterial extracorporeal membrane oxygenation using radial near-infrared spectroscopy.
Critical care (London, England)Crossed sympathetic pathways in 3 cases of Harlequin syndrome.
Revue neurologiqueHarlequin Syndrome and Autonomic Seizures - A Rare Association.
Annals of Indian Academy of NeurologyHarlequin syndrome in children: secondary until proven otherwise.
BMJ case reportsVeno-Arterial Extracorporeal Membrane Oxygenation for Cardiogenic Shock.
Journal of visualized experiments : JoVESarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report.
Medicina (Kaunas, Lithuania)"Partial" Harlequin Syndrome in Central Veno-Arterial Extracorporeal Membrane Oxygenation.
Journal of cardiothoracic and vascular anesthesiaIdiopathic Harlequin Syndrome in a Patient from Ethiopia: A Case Report.
Ethiopian journal of health sciencesExtracorporeal Membrane Oxygenation.
Respiratory careHarlequin syndrome during peripheral cardiopulmonary bypass in a patient with an obstructing tracheal schwannoma: A case report.
Clinical case reportsSuccessful management of harlequin syndrome due to pulmonary hemorrhage and atelectasis with VAV- ECMO.
PerfusionPediatric COVID-19 extracorporeal membrane oxygenation transport during the pandemic.
PerfusionHarlequin Syndrome Related to Perioperative Intercostal Blockade: A Case Report.
A&A practiceManagement of harlequin syndrome under ECPELLA support: A report of two cases and a proposed approach.
Annals of cardiac anaesthesiaVideo-assisted thoracoscopic sympathectomy for Harlequin syndrome.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryThe effect of arterial cannula tip position on differential hypoxemia during venoarterial extracorporeal membrane oxygenation.
Physical and engineering sciences in medicineHarlequin syndrome as a complication of epidural anaesthesia in an infant: Do adjunct medications play a role?
Indian journal of anaesthesiaHarlequin Syndrome Following Regional Liposomal Bupivacaine Use in a Partial Sternectomy.
CureusHypothermic Cardiac Arrest Managed Successfully by Changing ECMO Configurations.
Journal of cardiothoracic and vascular anesthesiaHarlequin syndrome induced by intraspinal analgesia in patients with advanced cancer: a case report.
Translational cancer researchMini-Review on the Harlequin Syndrome-A Rare Dysautonomic Manifestation Requiring Attention.
Medicina (Kaunas, Lithuania)A novel SCN9A gene variant identified in a Chinese girl with paroxysmal extreme pain disorder (PEPD): a rare case report.
BMC medical genomicsHarlequin Syndrome: Discovery of an Ancient Schwannoma.
CutisHidden Harlequin syndrome in neonatal and pediatric VA-ECMO.
Critical care (London, England)Harlequin syndrome: An asymmetric face.
Clinical case reportsHarlequin syndrome associated with thoracic epidural anaesthesia.
Anaesthesia reportsSCN10A variants associated with congenital harlequin syndrome.
The British journal of dermatologyHarlequin Syndrome, Apnea, and Acute on Chronic Hemiparesis: An Atypical Pediatric Case of Chiari I With Holocord Syringohydromyelia.
Pediatric neurologySuccessful treatment of idiopathic Harlequin Syndrome with oxybutynin and propranolol.
The Australasian journal of dermatologyHarlequin syndrome after surgery for aortic dissection.
Intensive care medicineCase Report: Left Ventricular Unloading Using a Mechanical CPR Device in a Prolonged Accidental Hypothermic Cardiac Arrest Treated by VA-ECMO - a Novel Approach.
Frontiers in cardiovascular medicineHarlequin syndrome in a young girl: An opportunity to rethink the terminology.
Pediatric dermatologyManaging Harlequin Syndrome in VA-ECMO - do not forget the right ventricle.
PerfusionHarlequin syndrome with petechiae caused by a pituitary adenoma.
Journal of the European Academy of Dermatology and Venereology : JEADVHarlequin syndrome in a pediatric population: a case series.
Acta neurologica BelgicaMust be sympathetic nervous system with unilateral facial flushing in harlequin syndrome.
Lancet (London, England)Peripheral Cardiopulmonary Bypass in Two Patients With Symptomatic Tracheal Masses: Perioperative Challenges.
Journal of cardiothoracic and vascular anesthesiaHarlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryBilateral harlequin syndrome, unilateral Horner syndrome, and Riga-Fede disease as presenting features of hereditary sensory and autonomic neuropathy type IV.
Pediatric dermatologyHarlequin syndrome following microwave ablation in a child with a symptomatic paraspinal mass.
BMJ case reportsRisk of harlequin syndrome during bi-femoral peripheral VA-ECMO: should we pay more attention to the watershed or try to change the venous cannulation site?
Critical care (London, England)Patient with Cluster Headache and Harlequin Sign - Related or Not?
HeadacheParoxysmal extreme pain disorder in family with c.3892G > T (p.Val1298Phe) in the SCN9A gene mutation - case report.
BMC neurologyA novel veno-arteriovenous extracorporeal membrane oxygenation with double pump for the treatment of Harlequin syndrome.
PerfusionDetection of subclinical Harlequin syndrome in pediatric patients.
Paediatric anaesthesiaHemifacial erythema and sweating after exercise in a child.
Pediatric dermatologyHarlequin Color Change in a Neonate.
The New England journal of medicineHarlequin syndrome caused by isolated focal parasympathetic neuropathy.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyHarlequin sign in Pancoast tumor.
Current problems in cancer[Harlequin syndrome after scoliosis surgery].
Der OrthopadeNorth-south syndrome in veno-arterial extra-corporeal membrane oxygenator: the other Harlequin syndrome.
Canadian journal of anaesthesia = Journal canadien d'anesthesieHarlequin Syndrome after Thoracoscopic Repair of a Child with Tracheoesophageal Fistula (TEF).
European journal of pediatric surgery reportsIdiopathic harlequin syndrome: a case report and literature review.
The Pan African medical journalCardiac Sympathetic Hyperactivity of Lung Cancer-Associated Harlequin Syndrome.
JMA journalPure autonomic failure presenting as Harlequin syndrome.
Autonomic neuroscience : basic & clinicalHarlequin Syndrome in Acute Thalamic Hemorrhage.
Journal of stroke and cerebrovascular diseases : the official journal of National Stroke AssociationImage Gallery: A case of Harlequin and Horner syndromes.
The British journal of dermatologyHarlequin Syndrome during Venoarterial Extracorporeal Membrane Oxygenation.
Radiology. Cardiothoracic imagingCross-Field Ventilation for Treatment of Harlequin Syndrome in Traumatic Tracheobronchial Injury Repair Using Intraoperative Venoarterial Extracorporeal Membrane Oxygenation.
Journal of cardiothoracic and vascular anesthesiaHarlequin Syndrome Associated with Erector Spinae Plane Block.
AnesthesiologyNeurologic aspects of sweating and its disorders.
NeurologyBranch Retinal Vein Occlusion in Ipsilateral Harlequin Syndrome.
Klinische Monatsblatter fur AugenheilkundeUnilateral Anhidrosis With Ipsilateral Basal Cell Carcinomas.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Unconventional Cannulation Strategy in Peripheral Extracorporeal Membrane Oxygenation to Achieve Central Perfusion and Prevent Differential Hypoxia.
Journal of cardiothoracic and vascular anesthesiaChild Neurology: An infant with episodic facial flushing: A rare case and review of congenital harlequin syndrome.
NeurologyDo you know this syndrome? Harlequin syndrome.
Anais brasileiros de dermatologia[Harlequin syndrome secondary to a neuroblastoma].
Anales de pediatriaPosition of draining venous cannula in extracorporeal membrane oxygenation for respiratory and respiratory/circulatory support in adult patients.
Critical care (London, England)Postoperative Harlequin Syndrome: Case Report of a Rare but Clinically Striking Condition.
A&A practice[Harlequin syndrome: Two sides of the same disease].
SemergenHarlequin syndrome following upper lobectomy.
Asian cardiovascular & thoracic annalsIsolated Harlequin Syndrome Following Brachial Plexus Nerve Block via Interscalene Approach: A Case Report.
AANA journalHarlequin syndrome as a rare complication after epidural anesthesia in an obstetric patient.
Minerva anestesiologicaControlled flow diversion in hybrid venoarterial-venous extracorporeal membrane oxygenation.
Interactive cardiovascular and thoracic surgeryHarlequin syndrome and Horner syndrome after neck schwannoma excision in a pediatric patient: A case report.
MedicineDevelopment of Harlequin Syndrome following placement of thoracic epidural anesthesia in a pediatric patient undergoing Nuss procedure.
Clinical case reportsUnilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome.
Indian journal of dermatology, venereology and leprologyThree Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula.
Annals of vascular surgeryHarlequin Syndrome Following Resection of Mediastinal Ganglioneuroma.
The Korean journal of thoracic and cardiovascular surgeryIdiopathic Harlequin Syndrome Manifesting during Exercise: A Case Report and Review of the Literature.
Case reports in medicineHarlequin sign concomitant with Horner syndrome after anterior cervical discectomy: a case of intrusion into the cervical sympathetic system.
Journal of neurosurgery. SpineCongenital Cases of Concomitant Harlequin and Horner Syndromes.
The Journal of pediatricsHarlequin Syndrome Associated with Multilevel Intercostal Nerve Block.
AnesthesiologyHarlequin syndrome with contralateral anhidrosis after an upper chest gunshot wound.
BMJ case reportsHarlequin Syndrome after Stellate Ganglia Neuroblastoma Resection.
The Journal of pediatricsHarlequin syndrome in a paediatric patient: A diagnostic challenge.
NeurologiaHarlequin Syndrome in Cluster Headache.
HeadacheVenous Cannula Positioning in Arterial Deoxygenation During Veno-Arterial Extracorporeal Membrane Oxygenation-A Simulation Study and Case Report.
Artificial organsA Case of Undiagnosed Harlequin Syndrome Presenting in General Dental Practice.
Dental update[Harlequin phenomenon associated with neurological abnormalities: A case report].
Annales de dermatologie et de venereologie[Harlequin syndrome in a melanoderm patient].
Revue de stomatologie, de chirurgie maxillo-faciale et de chirurgie oraleCongenital Harlequin syndrome as an isolated phenomenon: A case report and review of the literature.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyHarlequin color change in preterm infant.
The Pan African medical journalPitfalls in percutaneous ECMO cannulation.
Heart, lung and vesselsBotulinum toxin for treatment of Harlequin syndrome.
Parkinsonism & related disordersHarlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy.
Autonomic neuroscience : basic & clinicalUnusual pattern of neonatal hyperhidrosis.
BMJ case reportsHarlequin Syndrome After Thoracic Paravertebral Block.
A & A case reportsHarlequin Syndrome Following Implantation of Intrathecal Pumps: A Case Series.
Neuromodulation : journal of the International Neuromodulation SocietyHarlequin Syndrome Associated with Thoracic Epidural Analgesia.
AnesthesiologyHarlequin syndrome: a mask of rare dysautonomic syndromes.
Dermatology online journal[A woman with unilateral flushing of her face].
Nederlands tijdschrift voor geneeskundeHorner's, Heterochromia, and Harlequins.
Journal of general internal medicineIatrogenic harlequin syndrome: a new case.
Annals of dermatology[Evaluation of sudomotor function].
Rinsho shinkeigaku = Clinical neurologyConcurrent Horner's and Harlequin syndromes.
International journal of oral and maxillofacial surgeryHemifacial Erythema in Right Unilateral Electroconvulsive Therapy.
The journal of ECTAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Laser therapy: palliative care for the Harlequin syndrome?
- A 17-year-old boy with hemifacial flushing and anhidrosis.
- Case report of a cardiac Harlequin syndrome-electrical storm during venoarterial extracorporeal membrane oxygenation.
- Harlequin syndrome as part of cervicothoracic spine injury following a fall.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2025· PMID 40751882mais citado
- Routine Screening for Neuroblastoma in Harlequin Syndrome Without Otherwise Known Etiology.
- Harlequin Syndrome with Ipsilateral Facial Flushing After Internal Carotid Artery Dissection: An Atypical Sympathetic Laterality Pattern.
- Harlequin syndrome: segmental autonomic dysfunction in the realm of Sjogren's disease.
- A pediatric case of idiopathic harlequin syndrome.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:199282(Orphanet)
- MONDO:0016040(MONDO)
- GARD:8610(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q5658687(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
