What is Harlequin Syndrome?

Harlequin syndrome is a medical condition that affects the autonomic nervous system, meaning it is involuntary. It is signaled by sweating and red flushing on only one side of the face, often called the harlequin sign. It is a rare disease that was unnamed until doctors encountered it in a group of patients in 1988. Treatment is possible in some patients, but the condition without treatment is not normally fatal.

Typically, in cases of harlequin syndrome one side of the face does not sweat or flush at all. The other side of the face, however, flushes and produces a massive of amount of perspiration. In most cases, the sweat is so profuse that it drips down to the person’s arm, soaking it. This is all done involuntarily, 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 not known. In other cases, nerve damage was experienced at some point by the person, and that damage may signal a cause for the disease. Some people who suffer from tumors or strokes may experience the symptoms of harlequin syndrome as well. Usually, the face of the person affected by the disease will sweat and flush suddenly, 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. Repair is typically done surgically, by a skilled physician. If surgery is not possible, a patient may undergo a repetitive stellate ganglion block. This medical procedure uses a local anesthetic and has been proven to lower the severity and the duration of harlequin syndrome. Research publications have indicated that these techniques, although rather 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 may cause some embarrassment, but as people become used to the condition, those feelings should pass. Scientists constantly search for new ways to treat nerve damage and damage to the autonomic nervous system. As a result, people are hopeful that researchers will develop new ways to treat people affected by this rare disorder.