What Is Harlequin Syndrome?

Harlequin syndrome is a condition that affects the autonomic nervous system, and therefore occurs involuntarily. It is manifested as sweating and flushing on one side of the face, often known as the harlequin sign. It is a relatively rare disease that was unknown and unidentified until it was seen in a group of patients in 1988.

Treatment is probable only in some patients, however, the ailment is not normally fatal is treatment is not possible.

Characteristically, in harlequin syndrome, one side of the face flushes and produces an enormous of amount of perspiration, whilst the other side neither flushes nor sweats. In a majority of cases, the sweating is so profuse, that it drips down on to the individual’s arm, soaking it. This happens involuntarily, i.e. without the person’s capacity to control it. By and large, Harlequin Syndrome is the consequence of some form of nerve damage.

Harlequin Syndrome Symptoms

The condition is typified by sweating, profuse perspiration and great flushing on one side of the body, more often than not, the chest, the neck, and the face. The other half does not demonstrate any sweating or flushing at all. The symptoms occur involuntarily. They occur in response to an injury / trauma to the sympathetic nervous system, i.e. that part that usually responds to stress.

Harlequin Syndrome Causes

The following are the commonly seen causes for the harlequin syndrome:

• The symptoms frequently occur due to an injury to the sympathetic nervous system, that division of the nervous system that is responsive to stress.

• It may also occur after a unilateral endoscopic thoracic sympathectomy (ETS) or an endoscopic sympathetic blockade (ESB) surgery.

• Those who suffer from stroke or tumors may experience the symptoms of harlequin syndrome as well. Characteristically, the face of the person flushes suddenly and there is copious perspiration, without any warning or identified cause.

• From time to time, the symptoms of the disease may appear when the person exercises or spends too much time in the heat.

Harlequin Syndrome Treatment

When the surgeon can identify the damaged nerve, then the damage / impairment can be repaired, and treatment may be possible. Repair is done surgically. In case surgery is not possible, a stellate ganglion block is done. In this medical procedure, a local anesthetic is given, and it has been proven that it can diminish the intensity and the duration of the harlequin syndrome manifestations. Research indicates that these techniques, though rather new, are the most effective ways to manage the condition.

Prognosis of the harlequin syndrome is moderate to poor. The condition is generally not life threatening or fraught with serious complications, but it can cause awkwardness and discomfort.

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