What is Harlequin Syndrome?: Treating Harlequin Syndrome
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What is Harlequin Syndrome?: Treating Harlequin Syndrome

A rare disorder, Harlequin syndrome is often associated with profuse sweating and flushing of the body. Fortunately the condition is not associated with serious complications and can be managed, though the outcome of the condition is not good.

Harlequin syndrome is a relatively rare disorder which afflicts the ANS or the autonomic nervous system. The disorder is seen as flushing and extreme perspiration on half side of the body. The condition was unknown and remained anonymous until 1988, when it was observed in some patients.

Treatment is feasible only in a few patients, nevertheless, the disorder is not life threatening or fatal.

Typically, in the syndrome, one half of the body sweats profusely and flushes, while, the other portion does not sweat or become flushed. The symptoms appear involuntarily, i.e. one has no control over them and they come on all of a sudden.

What Causes Harlequin Syndrome?

The following are the common causative factors for harlequin syndrome:

  • Trauma or injury to the Sympathetic Nervous System, (that part of the nervous system that responds to stress) can bring on the symptoms of Harlequin syndrome.
  • It may be seen as a side effect to endoscopic sympathetic blockade or unilateral endoscopic thoracic sympathectomy surgical intervention.
  • Individuals who have tumors or have had stroke may experience the features of harlequin syndrome as well. Normally, the face becomes red and flushed all of a sudden and there is profuse perspiration, without any identifiable cause.
  • Now and then, the symptoms may appear when the person exercises or is in hot weather conditions for too long.

What are the Symptoms of Harlequin Syndrome?

Harlequin syndrome is characterized by profuse sweating and flushing on one side of the body, usually, the face, neck, and the chest. The other side of the body is normal and shows no flushing or sweating at all. The clinical features of the syndrome occur involuntarily.

Harlequin Syndrome Treatment

There isn’t any specific treatment regimen for the condition. Surgical intervention is recommended is certain cases. When it’s possible for the doctor to spot the impaired nerve, then the damage can be rectified. In case surgical intervention is not feasible, a stellate ganglion block is conducted. For the stellate ganglion block, a local anesthetic is administered; it has been proved that this technique can reduce the duration and the intensity of the symptoms of the harlequin syndrome. Though this technique is rather new, is it quite effective in the management of the disorder.

Outcome of a case of Harlequin Syndrome is moderate to poor. The disorder does cause discomfort and awkwardness, but, it is not fraught with any grave complications.

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Comments (4)

An interesting article on Harlequin syndrome.It would have been more useful if you had explained what is meant by 'stellate ganglion block'.

Very interesting article! This disorder sounds like it's not much fun to have.

Thanks for sharing this. This is something new to me.

I have Harlequin Syndrome and I am finding that I am afraid to go out in company because of the symptoms. I cannot get a doctor to help me and I wondered if you had any information on specialists in the UK.