Have you ever seen, or experienced, one side of your face suddenly turning red and sweaty, while the other side is completely normal? For some, this can be a strange, perhaps even uncomfortable experience. This is what we call Harlequin Syndrome in medical terms. While the name may sound like a big deal, it's nothing to be afraid of, but it's important to be aware of it.
What exactly is this (Harlequin Syndrome)?
Simply put, Harlequin Syndrome is a condition where only one side of your face, neck, and sometimes upper chest becomes red and sweaty . Surprisingly, none of this happens on the other side of your body, which remains normal.
This condition occurs due to a change in the functioning of our body's sympathetic nervous system . Now you may be wondering what this sympathetic nervous system is. Our body has a very amazing control system, called the autonomic nervous system . This is what controls many things that happen "automatically" without us thinking about them, such as our heart rate, blood pressure, breathing, and digestion.
Part of this autonomic nervous system is the sympathetic nervous system. It is like our body's 'alert' system . This system is activated when we run, when we are scared, or when we feel a strong emotion such as great joy or sadness. This is called the 'fight-or-flight' response . This sympathetic nervous system is the one that signals our body to sweat to control our body temperature, just like when we exercise to cool down. (Harlequin Syndrome) Symptoms appear when this sympathetic nervous system is activated.
Are (Harlequin Syndrome) and (Harlequin Sign) two different things?
Yes, there is a slight difference. (Harlequin Syndrome) refers to the neurological condition I mentioned earlier. (Harlequin Sign) refers to the main symptom of the disease. That is, the skin color on one side of the body changes (redness or darkening) and the skin on the other side remains pale or normal. This (Harlequin Sign) can be seen not only in (Harlequin Syndrome) but also as a symptom of some other health conditions.
Is this a very common condition? Is it life-threatening?
Harlequin Syndrome is actually a very rare condition. It has only been reported in a handful of cases worldwide. So it's not surprising if you haven't heard of it before.
The important thing is that this condition is not life-threatening . And it will not affect your lifespan. So there is no reason to be unnecessarily afraid.
What are the symptoms of this?
In Harlequin Syndrome, the symptoms you experience on each side of your body are different.
Symptoms that can be seen on the affected side:
- Skin turning red or darker than your normal skin color.
- Sweating a lot.
- The skin in that area feels warm when touched.
Symptoms seen on the unaffected side:
- Pale skin.
- Not sweating at all.
- The skin in that area feels cold when touched.
This way, these features look like the two sides of the body are divided in half. Imagine that you drew a vertical line from the middle of your forehead, down your nose, to your chest.
In addition to these main symptoms, some other less common symptoms may also be seen:
- In some places, the skin is dry and peeling like scales.
- Headache.
- Runny nose.
- Tears coming from the eyes.
- One-sided drooping of the eyelid and/or changes in the pupils (also called Horner Syndrome , which can be associated with a separate condition).
When do these symptoms appear?
You may experience these symptoms of Harlequin Syndrome, especially in the following situations:
- When exercising: When running, jumping, playing, or working out at the gym.
- During strong emotions or mental stress: when you are very happy, sad, scared, or under pressure before something like an exam or interview.
- In hot climates: when going out in the sun, when staying in a hot room.
- When eating spicy food: When eating a dish made with a lot of chili.
Imagine that you are playing cricket with your friends and you are sweating profusely, and only the right side of your face is red and sweaty, but the left side is fine, just like usual. That is what happens in this experience.
What are the causes of Harlequin Syndrome?
In most cases, it is difficult to find a specific cause for Harlequin Syndrome. That is, the cause is unclear. However, research has found that it may be caused by a malfunction in the communication between the cells on the right and left sides of our autonomic nervous system.
Sometimes, there can be a disruption in this communication pathway. This communication message travels from the hypothalamus in our brain to the thoracic spinal cord in the upper chest. That's where the sympathetic nerves supply the face and upper body. This pathway controls sweating and blood vessels.
There are several reasons why this interruption may occur:
- It is a tumor that develops along the nerve pathway.
- A wound or damage caused by an infection or inflammation.
- An accident that occurs due to surgery on the upper chest, such as a spinal operation.
Who is more likely to develop this? (Risk factors)
This condition, called Harlequin Syndrome, can affect anyone of any age, including infants, children, and adults . However, you may be more likely to develop this condition if you:
- If you have had an endoscopic thoracic sympathectomy (a surgical procedure used to treat excessive sweating).
- If you have received anesthesia.
- If you have had an accident that affected your sympathetic nervous system.
- If there is another underlying medical condition such as Horner Syndrome .
What complications can this cause?
Although this is very rare, some complications can occur due to Harlequin Syndrome. These are:
- Difficulty controlling body temperature.
- Permanent change in facial color (on one side).
- Depression, anxiety, or low self-esteem caused by thinking about one's appearance. Because, when others see this in society, it can be a little embarrassing for some people.
How to accurately diagnose (Harlequin Syndrome)? (Diagnosis)
A doctor will determine whether you have Harlequin Syndrome by examining your symptoms. The first step is a physical examination . Then, various tests are performed to rule out other conditions that may cause similar symptoms and to determine if there is an underlying cause.
There are several tests that can be used to diagnose Harlequin Syndrome:
- Stress test: In this test, a doctor will have you exercise and watch you to see if physical activity triggers symptoms. Harlequin Syndrome symptoms can be more pronounced during exercise.
- Imaging tests: These tests allow your doctor to see inside your body. These are painless tests. Examples include a chest X-ray, CT scan (Computed Tomography), and MRI (Magnetic Resonance Imaging) .
What are the treatments for this?
The good news is that Harlequin Syndrome doesn't always require treatment . Sometimes the condition can get better on its own. Whether a doctor recommends treatment depends on what's causing your symptoms. Treatment options include:
- Therapy or counseling can help reduce the social discomfort and mental stress caused by this condition.
- A shot given to control facial flushing or skin discoloration (stellate ganglion block) .
- Control sweating and other symptoms with botulinum toxin injections.
- If the cause is a tumor or lesion, surgery or radiation therapy will be needed to remove it.
What can you expect if you have Harlequin Syndrome?
Harlequin Syndrome usually doesn't cause any major harm to your body. Your symptoms may go away on their own over time. Or, you may only experience symptoms when you exercise or eat a spicy food. Not everyone needs treatment. Your doctor will tell you more about your condition and what to expect in the future.
How long do these symptoms last?
Symptoms usually last only a few minutes to a few hours . This means that after you stop the activity that caused the symptoms, the skin will return to normal within a short time. For example, if symptoms occur while exercising, once you stop exercising, cool down, and rest, the skin will return to normal.
Can Harlequin Syndrome be prevented?
As far as has been discovered so far, there is no way to prevent Harlequin Syndrome.
When should you see a doctor?
If you experience symptoms of Harlequin Syndrome, such as sweating on only one side of the body and changes in skin color, it is important to see a doctor . Harlequin Syndrome is usually nothing to worry about. However, in rare cases, it can be a sign of an underlying medical condition, such as a tumor, so it is important to see a doctor to determine the exact cause.
What questions should you ask the doctor?
When you go to see the doctor, it's a good idea to ask these questions:
- What is causing my symptoms?
- Do I need treatment or surgery?
- Are there any side effects of the treatment?
- If I feel embarrassed or uncomfortable because of my symptoms, what treatment options are available?
Are (Harlequin Syndrome) and (Harlequin Ichthyosis) the same thing?
No, these are two completely different situations. This is something that can confuse many people, so it's best to clarify.
Harlequin Syndrome is a condition that causes sweating and skin discoloration on one side of the body. It is not life-threatening, and it is not a genetic condition (however, in some cases it can be congenital).
On the other hand, Harlequin Ichthyosis is a life-threatening, genetic skin condition . A baby with this condition is born with very thick, rough skin. Babies with Harlequin Ichthyosis have diamond-shaped patches of skin that are separated by deep fissures. Sometimes doctors call this condition "Harlequin Baby Syndrome," which sounds similar to "Harlequin Syndrome," but these are very different conditions.
Take-Home Message
Harlequin Syndrome (a condition that causes temporary sweating and skin discoloration on one side of your body) affects only one side of your body. It's normal to feel uncomfortable when your skin looks different than usual. But remember, this is not a dangerous condition.
The most important thing is to see a doctor if you have these symptoms and find out the exact cause . Sometimes, the cause may not be obvious. Even if you say you don't need treatment, if you are experiencing any psychological discomfort due to this change in appearance, you can talk to a mental health counselor about it and talk about ways to build self-esteem and a positive self-image. You are not alone, and never hesitate to get help.
` Harlequin Syndrome, nervous system, sweating, flushing, autonomic nervous system, Horner Syndrome, rare diseases
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