Harlequin Sign

What is the harlequin sign?

The Harlequin sign occurs when you have color changes (redness or darkening) on one side of your face and body. The other side remains unchanged or appears pale. The sides of your body separate straight down the middle. This can appear as a vertical line (up and down) from the top of your head down through your chest.

Along with skin tone differences, you may experience other symptoms on each side of your body. On the red or darkened side, you may experience excessive sweating (hyperhidrosis) and your skin may feel warm to the touch. On the pale or unaffected side of your body, there won’t be any signs of sweat and your skin will be cold to the touch.

The Harlequin sign, or Harlequin color change, may be a symptom of certain health conditions, including Harlequin syndrome, but it’s typically harmless (benign).

What are the most common causes of the Harlequin sign?

Healthcare providers don’t always know the cause of the Harlequin sign. It may occur because of a miscommunication between the cells on the left and right sides of your body in your autonomic nervous system. There may be a blockage on a cell’s communication pathway. This is the path a cell’s message takes from your brain (hypothalamus) to your spinal cord. The cell’s message goes out to the nerves that provide your sympathetic nerve supply to your face and upper body. These nerves control your blood vessels and sweating. The blockage could also occur for various reasons.

Harlequin sign in newborns

According to research, the Harlequin sign occurs in about 10% of all healthy newborns. The Harlequin color change typically happens when a newborn is between two and five days old. But some studies note the occurrence as happening as late as three weeks old. The newborn Harlequin sign is harmless. The color change fades away in as little as 30 seconds. Sometimes, it takes 20 minutes for the color to fade. It may happen again when your infant is placed on their side.

The Harlequin sign in newborns isn’t the same thing as a life-threatening condition known as Harlequin ichthyosis. With this condition, large plate-like scales with cracks (fissures) cover your newborn’s body.

Harlequin syndrome

Harlequin syndrome is a condition that affects your sympathetic nervous system. It causes one side of your face and body to flush and sweat, while the other side doesn’t. Your sympathetic nervous system is part of your autonomic nervous system, which controls many of your body’s “automatic” functions like your breathing, heart rate, blood pressure and digestion.

Within your autonomic nervous system, your sympathetic nervous system oversees your “fight-or-flight” response. This activates when you feel strong emotions or your body is in motion. Your sympathetic nervous system helps control your body temperature by telling your body to sweat to cool down. With Harlequin syndrome, the Harlequin sign can appear whenever your sympathetic nervous system activates.

Horner syndrome

Horner syndrome is a rare neurological condition that affects one eye and the surrounding tissue on one side of your face. Other names for the condition include oculosympathetic palsy and Bernard-Horner syndrome.

Horner syndrome occurs when something blocks the pathway of the sympathetic nerves that connect your brainstem to your face and eyes. These nerves control involuntary functions, like the dilation and constriction of the pupils of your eyes and sweating (perspiration). The condition can cause facial flushing on the affected side of your face.

How is the Harlequin sign treated?

Treatment depends on the Harlequin sign cause.

The Harlequin sign in newborns doesn’t require treatment. It will go away on its own within seconds to minutes.

Treatment for Harlequin syndrome isn’t always necessary either, as the condition may fade away on its own. If your healthcare provider thinks you need treatment, it will depend on the cause of your symptoms. Treatment may include:

• Stellate ganglion block injection to block skin color changes or flushing.
• Botulinum toxin injections to help manage your sweating.
• Radiation or surgery to remove a lesion or tumor.
• Counseling or therapy to help with any anxiety you’re feeling about how the condition affects the way your body looks.

Treatment for Horner syndrome may not be necessary unless you have pain or discomfort. Your healthcare provider will treat the underlying cause of the condition. Treatment can vary widely because the condition has many causes.

What are the possible complications or risks of not treating it?

The Harlequin sign is generally harmless, and it will usually go away on its own. But your healthcare provider may recommend treatment for Harlequin syndrome to prevent the following complications:

• Trouble controlling your body temperature.
• Permanent facial redness/discoloration.
• Low self-esteem or anxiety due to how your body looks.

Can the Harlequin sign be prevented?

Researchers don’t know why the Harlequin sign affects some people and not others, so they don’t know how to prevent the condition.

When should the Harlequin sign be treated by a healthcare provider?

You should visit a healthcare provider if you notice the Harlequin sign — redness/discoloration, sweating and warm skin on one side of your face and body. They’ll want to perform a physical exam and ask you about your symptoms. They may recommend certain tests to rule out other conditions. While rare, a tumor or another underlying condition could be causing the symptom. If you have a condition like Harlequin syndrome, your provider will talk to you about possible treatment options.