Harlequin syndrome, also known as Harlequin sign, is a rare neurological disorder characterized by sudden and dramatic changes in skin color and temperature on one side of the face and upper body.
Harlequin syndrome reflects following symptoms that makes us realize the problem –
• Unilateral flushing and sweating: One side of the face and neck turns red and becomes warm, while the other side remains normal.
• Anhidrosis: The red side may experience decreased sweating, leading to dryness and potential overheating.
• Constriction: The blood vessels on the affected side may constrict, causing asymmetrical blood flow and temperature regulation.
Harlequin syndrome is caused by dysfunction of the autonomic nervous system, specifically affecting the sympathetic nerves that control blood vessel dilation, sweating, and temperature regulation.
In some cases, the disorder may be idiopathic (of unknown cause), while in others it may result from trauma, surgery, or underlying conditions affecting the sympathetic nerves.
Harlequin syndrome is typically classified as primary (idiopathic) or secondary (resulting from an underlying condition).
• Primary Harlequin syndrome has no identifiable cause and is considered a distinct entity.
• Secondary Harlequin syndrome can be associated with conditions such as thoracic outlet syndrome, cervical rib abnormalities, or nerve injury.
Risk Factors –
While Harlequin syndrome is rare, it can affect individuals of any age.
There are no known specific risk factors, but certain underlying conditions or injuries to the sympathetic nerves may increase the likelihood of developing the syndrome.
Diagnosis is based on clinical presentation, symptoms, and medical history.
Tests may include thermoregulatory sweat testing, imaging studies (such as MRI), and nerve conduction studies to evaluate sympathetic nerve function.
Management focuses on addressing the underlying cause, if identified.
• Symptomatic relief can be achieved through measures like avoiding triggers, maintaining a cool environment, and staying hydrated.
• In some cases, medications that regulate the autonomic nervous system may be prescribed.
• Surgical interventions might be considered for secondary Harlequin syndrome if the underlying condition is amenable to treatment.
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