What is Harlequin syndrome?

Harlequin syndrome is a medical condition that affects the autonomic nervous system, which means that it is involuntary. It is marked with sweat and red flushing on only one side of the face, often called the sign of the harlequin. It is a rare disease that was not identified until doctors found it in a group of patients in 1988. Treatment is possible in some patients, but the condition without treatment is usually not fatal.

Usually in cases of harlequin syndrome, one side of the face does not sweat or blush at all. However, the other side of the face flushes and produces a great deal of perspiration. In most cases, the sweat is so profuse that it drips down the person's arm, drenching it. All of this is done unintentionally, or without the person's ability to control it. Most often, it is the result of some form of nerve damage.

In most cases, the exact cause of harlequin syndrome is unknown. In other cases, the person experienced nerve damage at some point, and that damage may indicate a cause of the disease. Some people who suffer from tumors or strokes may also experience the symptoms of Harlequin syndrome. Usually the face of the person affected by the disease will suddenly sweat and flush, without warning, while other times, the harlequin sign will appear because the person was exercising or spent too much time in the heat.

If the damaged nerve can be discovered and repaired, treatment may be possible. The repair is usually performed surgically, by a skilled physician. If surgery is not possible, a patient may experience a repetitive stellate node block. This medical procedure uses a local anesthetic and has been shown to reduce the severity and duration of Harlequin syndrome. Research publications have indicated that these techniques, while quite novel, are the best ways to treat the condition at this time.

As with any medical condition, people affected by Harlequin syndrome should have open communications with their doctors. In most cases, the disease is not life threatening. It can cause some embarrassment, but as people get used to the condition, those feelings should pass. Scientists are constantly looking for new ways to treat nerve damage and autonomic nervous system damage. As a result, people hope that researchers will develop new ways to treat people affected by this rare disorder.

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