Health

Harlequin Syndrome

The story of a startling reflection

February 11, 2010

While working out at the gym one afternoon in August of 2008, Francis Lam began feeling “sweaty and hot, and — kinda weird,” as though there was a cool breeze blowing only on the right side of his face.  “I look over and see that there’s sweat dripping down my left arm,” remembers Lam, “and my right arm is somehow totally, totally dry.”

In the locker room afterward, Lam glanced in the mirror to find a bizarre reflection staring back at him: his face was flushed deep red and sweating profusely — but only on the left side. “I looked like the jester or something. There was literally a line down the middle of my face,” he recalls. His left arm, too, was sweaty and florid, while his right was dry and pale.

The startling nature of Lam’s normally healthy thirty-year-old reflection was a sign of Harlequin syndrome, a rare and poorly understood disorder of the nervous system.

The symptoms vary from one individual to the next, but always include the “Harlequin Sign,” where one side of the face doesn’t sweat or flush at all, a condition known as anhidrosis. The effect is extremely pronounced because the other half of the face compensates by sweating and flushing excessively — a condition called hyperhidrosis.

Harlequin syndrome is a result of damage to the autonomic nervous system, the “unconscious” part of the nervous system that controls the involuntary muscle contractions of organs, gland activities such as sweating and crying, and the fight-or-flight response. Nerves carry instructions for these actions to different parts of the body, so damage to a single nerve can result in loss of function at its destination.

However, like most diseases of the central nervous system, cases of Harlequin syndrome are almost always “idiopathic,” meaning doctors don’t know what causes the nerve damage, says Dr. Peter Drummond, a neurologist at Murdoch University in Australia who first described the syndrome in 1988. “Our best guess is that the nerves are being attacked by a virus, but we really don’t know,” says Dr. Drummond.

Lam’s case may be one of the few with a discernible cause — a cause whose story is as unlikely and, well, double-sided as the condition itself.

One evening a few months before discovering his Harlequin syndrome, Lam, who works as a food writer in New York, was mugged and beaten while walking home to his Queens apartment. A trip to the emergency room revealed a mild fracture in his eye socket, as well as something Lam hadn’t seen coming: a chest x-ray showed a large, solid mass in his torso, above his right lung.

Several weeks of doctors’ visits and tests resulted in a diagnosis: a tennis-ball sized “schwannomma” — a benign tumor growing off of the second nerve in his thoracic spine. It would have to be removed.

When Lam noticed his strange sweating pattern a few months before the surgery to remove the tumor, Lam called his neurologist, Dr. Mark Bilsky, at Sloan-Kettering Memorial Hospital in New York. Dr. Bilsky told Lam that the growth of the tumor may be at fault, but added that some damage to the sympathetic nervous system would almost certainly occur during surgery, anyway, because the system is so sensitive that “if you look at it the wrong way it’ll screw up.”

In the fall of 2008, Lam’s doctors managed to remove his tumor without damaging the nerves responsible for motor control in his hand — which were wrapped around the growth like twine on a package. But symptoms of Harlequin syndrome have persisted.

Though natural nerve regeneration is possible, says Dr. Drummond, nerves sometimes grow back in “haphazard ways” that have strange manifestations. Sometimes sweat nerves reconnect with salivary nerves, resulting in “gustatory sweating,” in which the brain’s instructions to salivate are mistranslated into instructions to sweat.

But how could a tumor in Lam’s chest have caused a sweating condition in his face and arm?

The connection, explains Dr. Martin Duddy, a neurologist at Newcastle upon Tyne in the United Kingdom, lies in the unique arrangement of the sympathetic nervous system. The system is linked together in a chain that starts in the brain, at the hypothalamus, and progresses down the spinal cord. Nerves split off from the spinal cord through small holes in the vertebra.

The nerves that travel to the face from the spinal cord “actually track all the way down into the chest cavity and then make their way back up to the face,” Dr. Duddy explains, emerging from the spine around the same vertebra where Lam’s tumor was growing. Dr. Drummond notes that tumors in that location can put pressure on nerves, pinching them like a kink in a hose or, if the tumor grows large enough, disrupting the nerve altogether.

Whether or not the symptoms of Harlequin syndrome diminish, Lam feels lucky to have sustained so little physical damage throughout his trauma. Though his good sense of humor keeps him from fretting too much about the disorder, he still seems somewhat baffled by the strangeness of it. “My left arm sweats a lot,” he says, “but then I only sweat on the right side of my torso. I’ll look down and see that my shirt is totally soaked in this perfect square, but then the other side of my shirt will be completely dry. It’s weird.”

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Discussion

92 Comments

Joshua says:

hi. im having its symptoms. the right part of my body; my right leg, arm, back, abdomen, feet and neck are sweating nut the right part of my body is totally dry and pale.. i am so afraid.. what should i have to do?

didi says:

Hi, i am from Serbia, my 4 year old son has harlequin syndrome, from birth. To be precise we noticed it when he was 6 month baby. It affects his face and arm, face is practically divided in middle , one side is red and sweaty, and other pale and not a drop of sweat. Also his right hand is cold so in winter time he often cant go outside because he has pains in that hand. I was to a lots of doctors and they don’t know anything. I diagnosed him trough emailig with experts from overseas countries. My biggest problem is that i dont know what caused it. And because doctors in my country are idiots I dont know what to do and to who to turn for help. Reading some of this made me feel better..Tnx a lott for all comments.

Kathy says:

I am a 41 year old female, healthy. Last year I noticed half my face(left) turns red and feels warm on the touch after running on the treadmill for 45 minutes, especially in a warm day. I’ve had bad migraines since I was a teenage. I mentioned my condition to my doctor last year. She said she didn’t know what caused it and would look it up but haven’t heard from her.I work out 30 minutes every day and was afraid to work too hard. My doctor told me just to watch out.I have been looking up for more answers and found people here have the similar condition. Please post your comments if any one here find out more about this condition.

Maureen says:

Last year a friend told me I was only red on one side after jogging. Last week, I looked in the mirror after exercising and saw that I was red and sweating only on the right side. When I looked for those symptoms on the Internet, I found Harlequin’s syndrome. I think it is probably due to a syrinx that caused nerve damage in my spinal cord.

Tamra says:

I have MS. Lately during the day I have redness and heat on one side of my face. When I was younger my ear would get red and hot. I just wonder if my MS has something to do with this?

Mandee says:

I just had a baby 10 days ago & I noticed that when she cries sometimes her face will halfway go red. Like a perfect line right down the middle.. The pediatrician said birth mark. But is it possible she has this Harlequin Syndrome?

Thank you for enlightening us on what Harlequin Syndrome.I know this is a rare autonomic disorder of the nervous system, but is there a cure for this?

Tina says:

I too have been diagnosed with Harleyquins. My memory has been bad since the nerve damage.

Maryanne says:

Ellen Lensing Kitzman comment… I also have Harlequin sign (Horner’s Syndrome) and have been diagnosed with Lupus, strange to meet someone with the same diagnose as that! I have had the Harlequin sign for a very long time, possible at birth. It’s been a long haul of health issues for me. Your post was made a while ago but I’m really interesting in hearing more from you and the relation to the two diagnoses that we share.

Stephen says:

I am a male 58 years of age. Even now after 15 years of sweating profusely on the left side of my body when exercising or doing strenuous things and seeing my reflection in the mirror of a dividing line running down the centre of my face, red and wet to the left side and white and bone dry to the right side. I still get shocked by this. I have noticed my heart rate increases dramatically at this time. This also make me feel uncomfortable. I love going for walks in the country side, I never know what to wear. If it’s cold weather I can’t put to many warm clothes on because if I do the sweating starts a lots earlier on my left side. Then I get cold with the wet. Someone needs to invent a two sided jacket, warm like toast on the right and light and cool on the left, this might alleviate my problem when walking.
I have tried at my doctors to get help or even just information. I didn’t get any. I thought that I was the only person with this complaint till I came across scienceline.org when doing some research. I was amazed that there are lots more people with this condition.

carolyn says:

Carolyn
I have just read through everyone’s comments and can only say that I am another one of you. I too have suffered all the embarressing looks that can come our way because on the coolest of days when everyone around me is rugged up in coats and scarves and almost shivering, I am dripping sweat all down one side of my dual coloured face while my lovely hair do slowly crumples and lays in a wet flat and dripping heap on my head….but always/on one side only… and I can add to that picture a nose that drips water constantly so I sniff all the time like I see junkies doing. Or there is the constant ‘are you OK’ remark that comes from caring souls but that I find awfully embarressing. My clothes end up wet and i cant carry a changewith me everywhere i go…..and then I get cold and have to stay that way til I get home or til the next episode hits….. I just wish some doctor would discover something to even just help us live with this condition. I am becoming a hermit as I now hate going out anywhere…… I. Just hate the idea that this condition as to be with me……us……forever. thanks for letting me complain as I have and thanks for sharing your stories.
Carolyn

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Chris says:

Thank you all for sharing your stories. To my relief, an allergy specialist was able to give me a name (Harlequin Syndrome) for me to do my own research. It’s unfortunate there’s not enough information to do much about it. My hypothesis is that there was nerve damage due to either head injury during infancy or toxins in the environment or bacterial/viral infections to the nervous system. Can anyone confirm this hypothesis? Does anyone know where to turn for expert advice?

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Dedee says:

This site was so helpful, thank you. I had a chemical burn on my scale from a hair salon about 2 years ago. The burn was on the top left side close to the front. I was very sick for several months and on several different antibiotics. After a few days after the burn a huge lump moved down to my forward, like a egg. The lump moved down between my eyes, my eyes where swollen, I looked just like Avatar! After it or what ever it was continued to move down, I felt so sick. I woke up one night and threw up, it was a green so colors. Once I threw up I started to feel better but I still don’t feel right. My face turns bright red on my right side after a bath, a straight line in the center of my face and it’s just my face. I have a surging feelings from the back of my head at the crown every once in a while. I also have been having nerve problems from my knees running up the inside of my thighs to the front of my thighs at the top. I have seen a change in many parts of my body but mostly in my lower back and digestive system. I have always been healthy and very active, 5’2″ 109 lbs. I took self pictures of my burn almost daily. Keep it clean without any products on it. A plug of hair came out about 2 months after the burn. I called the salon, thats another story in its self. I called L’Oreal , they said it was not their problem even though it was their product, it was the salons responsibility . I am concerned about a product like this being tested on Animals and being used on humans. I have not be able to have a MRI, my insurance deductible is 3,500. My face started turning red on the right side after this happened
Could a burn like this be the cause. I would appreciate and thoughts on this. Thank you

lalit Maura says:

Hi, since last two years or so my son, aged 15 now, was complaining about half red and sweaty face while other half cool, particularly after a play. I didn’t gave much attention to it. However, one weekly off day, I was at home and I saw a clear cut line running down his face dividing it exactly in two halves! reddish on one side and pale on the other! He had just returned from a cricket match. After sometimes it was normal. He is a healthy boy without any problem till now. My wife continuously asked me to visit a doctor for this problem. So today, before going to a neurologist, I searched for it in internet and found this site. From this, I came to know that he too has a harlequin syndrome! I don’t remember any incidence for which he has this syndrome. Of course, that much I remember that he had a small eye compare to other one during his early childhood. But now there is no difference in his eye sizes. I am relived as most of the commented stories tell it is not a symptoms of any dangerous disease. I am certainly going to visit a neurologist for this in future. Thanks.

MIRA says:

hi, im 16 years old, and I don’t if it is really a harlequin syndrome but most of the patience experience I experience it too, is it possible at my young age have this syndrome.??

Darleen says:

In Dec. 2014 I found the page on facebook: Ross, Harlequin, Holmes-Adie and Horner’s Syndrome… where I was able to find out that I had Harlequin Syndrome for the last 20 years! First time I read that there are other people in the world that have what I have. Great site. Now I found this site. So very useful to those of us where the doctors have no idea what we have! Thank you and good luck all. I am just living with it.

nikki says:

and after 10 years I finally found the answer. I’ve asked this to the doctor, but he said it was not anything. whether this is related to heart disease? because my symptoms followed by symptoms of heart disease

nikki says:

This when I was 13, but it sometimes appears.

Adam Record says:

I have the same thing
I am 43 years old and scared. Glad to hear I am not the onlyone with this.
Can I die from this, the Drs. shour dont know what to tell me.I went to the best hospital in Boston ma.I got pissed of and walked out after 6 days with out any answers.
If someone knows a Dr that knows what the hell is going on with me please give me a call 978-270-9742 .I don’t know how to us my e mail so pleas call me thanks

Mara says:

I’ve had Drs treat me as tho I am nuts. Finally I happened to be maybe 5 minutes from one Dr’s office so I stopped in, and after the girl at the front desk took a long shocked look at my face, she asked me to wait and my Dr was out to take a look immediately! She looked and touched and finally said she had never seen anything like it before, and asked me if I could have had a stroke at some point. Not that I was aware of, after which she had run out of ideas. Period. But, at least she knew it wasn’t my imagination. Since then I have searched the internet with no luck at all-I must have just not chosen my words right because today I tried again and almost cried as I read case after case, in fact 68 of them, describing what I’ve been dealing with for at least 20 years! And it has a name!!! So my question is, have any of you put together a website or someplace to compare symptoms and commonalities? I read one person had Lupus, I have Fibromyalgia for starters, and a whole pathetic list of diagnosis to go along with it. But if there is a site where everyone is contributing information, I would be very interested in participating. Thanks for allowing me to rant my fellow Harlequin-ites!

Molly says:

I am so glad to have found this article and the related comments! I have a 3 year old who has had the harlequin discoloration since birth. It now covers slightly less of his trunk, but when he is either cold, hot, or upset, the left side of his trunk and his left arm turn red and there is definite line separating the 2 sides of his body. He just had an MRI of his cervical spine and brain which came back normal. I am wondering if we should look into having an MRI of his thoracic spine as well. The appearance doesn’t bother me, but I am concerned about any other ways it is currently or may affect his health in the future. Anybody else have this beginning at birth and persisting since then?

Karen says:

This happened to me five days before I had a stroke. Not sure if it was related.

I’m a seventeen-year-old senior. I’ve had this for years and started noticing it especially when I played soccer and got all sweaty. Only the right side of my face gets hot and red and the left side is cool and white. There’s a perfect line down my face when this happens. Also, only the right side of my forehead sweats. I’ve noticed this happening to my arms and maybe my hands in the past, where one hand will be dry and more chapped, and the right one will be sweaty and I can grip things a lot better with it. I’ve had two major surgeries and am almost certain that’s what caused the nerve damage. I recently just found out that this condition has a name. I thought I was the only one! I’m so relieved that there are other people who actually know what it’s like. We are all beautiful, unique individuals!

Alex Campos says:

I was diagnose with Harlequin as a baby also I have different colored eyes my left eye has an outer rim of blue (it changes) and goes into green and light hazel in the middle like layers and my other eye is brown, and for some reason my eyes are different sizes my left one appears to be more “asian looking” but it is not noticable if I do not pointed out and i get a line in the middle of my stomach although I do sweat from my whole body the only part I have never sweated from is the left side of my face. My family thinks that the smaller eye comes from my mother and the larger one from my father I have never met another person in my life that suffers from Harlequin and I am very active I wrestled in high school as well as football and soccer and i go to the gym right now this sindrome does not affect my everyday life in fact I think it is a really unique trait to have.

Colin Marson says:

Lots of familiar stuff here. I had thoracic surgery to remove a Schwannoma, about the size of a tennis ball, in 1985 when I was 23 years old. Twelve months later on heading for an overseas holiday I went to see a sports doctor about my inability to sweat on the left side of the head, upper body and left arm, and it often getting painfully cold during running in cold weather. Diagnosis was Horner’s Syndrome caused by the tumour. Should have gone to see a doctor many years earlier when I first noticed the symptons! Have since gone on to run okay, 32mins for 10km, 2:36 for a marathon, just need to learn to live with the body temperature issues – heavy sweating in warm/humid weather, and layer up on the left when it’s cold, especially important when cycling, make sure you wear decent gloves so you can use the brakes! Also when I exercise for a long time outdoors in cold weather I often suffer from speech issues, left side of the head/face freezes up. And like a few other have commented I’m unable to grip smooth objects properly due to lack of moisture in the hand. Have some pain issues still from the surgery around the scar, think it may be where the scar tissue crosses the sympathetic nerve “boundaries’. On the plus side, a couple of times during races in hot weather I would position my “dry” side next to the competition and then ask them why they were sweating so hard…

Kim Kent says:

It feels good to know that I am not the only person suffering with this. I noticed that I only sweat on my right side several years ago. I don’t remember having this problem when I was a child. I probably noticed it in my early to mid thirties. I am now 49 years old. I have talked to my family doctor and my neurologist about it, but neither of them could give me a diagnosis. Within the last two years it has really affected my quality of life. I avoid going anywhere where I know I will overheat such as outdoor events in the summer, shopping centers, crowded areas. At work I have to have a fan blowing on me all of the time or I will be drenched in sweat only on my right side. My hair will be dripping wet, sweat will be dripping off from me, my clothes will become drenched with sweat only on the right side of my body. My left side will be completely dry. I look freakish when it happens. As a teacher my students will often ask if I am okay when they see the crazy amount of sweating and the red and purple color of my face. As I am approaching menopause my condition is worsening as I feel like I am in the throes of hot flashes most of the time. My doctors can’t explain it and I feel as if they don’t comprehend just how bad it is. I wish there was some treatment that could help me. I can’t imagine living like this for years to come.

Rita Gurski says:

I’m a 75 year old female born in Germany. At an early age my mother noticed that when I got hot, my face had a straight line down the middle with one side white and the other red. She mentioned that my great grand mother had the same issue so I did not pay much attention to it. However, lately I also noticed that one side of my face has gotten very wrinkly and the other side is relatively smooth. When I perspire one side is damp with moisture and the other side is totally dry. I further noticed that my nails on my hands grow differently, longer on one hand and shorter on the other. I also have scoliosis, I wonder if that has something to do with my Harlequin disease?

Claire Dodd says:

I’m almost 22 and I started experiencing this syndrome about 7 or 8 years ago. I’ve played sports all my life but at the time, I was playing a lot of volleyball. Only half my face gets red and sweaty, and only have my head gets sweaty as well. So one half will be drenched in sweat, then I turn around and the other side is sweat-free. It’s crazy. Funny that so many have experienced sweat only on one arm; I sweat on both. I’m also not that affected by the cold like many have previously stated.

Bankole rasak says:

For about 2years now have been noticed my body we divided into 2equal parts,one we be sweating from neck to foot and other we be completely dry from neck to foot.

Whisper says:

I now have this on my face after going through radiotherapy for breast cancer. The left side stays pale and the right red and sweaty with a very straight line down the centre of my face. It does knock your confidence a bit. But I think if that’s what I have to put up with but I’m still alive then hold my head high and ignore people looking..

Tammy says:

I have had this condition for about 10 years. First noticed it after working in the garden in the heat of the day. It only affects my face. I train for endurance events and have never had an emergency due to this condition. I do have very low blood pressure and occasionally suffer from dizziness but I do not believe the two are related.

Marney says:

It is very interesting to find out about this condition. Thanks to everyone for sharing their stories. I am a t5 complete paraplegic since 1985 but since about 2005 where i had back surgery to install some titanium rods (t1 down to l1) i have had this uneven & sometimes profuse sweating & clamminess on my left side. During exercise i only need to wipe the left side of my face as an example. Just a few days ago i looked in the mirror & saw the white/red difference straight down the middle of my face, very noticeable on the forehead, told my husband when i got home from the gym & now reading this is very interesting, going to discuss with my family doctor.
Oh ysa i return to my back surgery, i thought that the surgeon maybe cut into some nerves that affect sweating & as i haven’t seen him since, never got to ask him about it.
Having more & more nerve pain, neck pain, etc so i think it might be a good time to call the surgeon & get a check up. Unfortunately most family docs here dont know much about paraplegia due to lack of training (a 2 hour lecture is all they get) and not very much experience in their general experience. What can he do, the system /education needs to improve unless all SCI people go to the same GP, yeah right. Will test his knowledge on harlequin syndrome :)
Well thanks again, great forum, very helpful. Good luck to all who are suffering, hope their will be help for us all soon, God Bless.

Dr. Sham Ramnarine says:

I am 70 years old. Today is the 5 January 2017. On 29 December 2016, I developed flu-like symptoms – fever, vomiting, cough, lower back pains, diarrhea – which I contracted from my 8 year old grandson whom I looked after during his illness. I took paracetamol, cough syrup and rehydrating fluids and rest in bed and sitting. Three days ago, I noticed that my left thigh and leg became wet and cold while the rest of my body remained normal. I thought that may have been a weird reaction to the paracetamol. However, this has recurred for the past three days even though I have stopped taking the paracetamol. I am wondering if this is a variant of Horner’s syndrome.

Ravi Rathod says:

Right side of my stomach is sweating and the other side is clear. I was suffering from Fever yesterday night and today at morning I went to the doctor and after that this sweating got started. What to do ?

Christine says:

My 14 year old niece was just diagnosed with pneumonia and she’s disabled her forehead is cool her cheeks a bright bright red and hot and her cavity is cold and clammy is this something that we should be worried about

Fran says:

25 yrs ago I had lung surgery on my left side. The tumor was a nerve tumor. They went in to remove the tumor and top half of left lung. Tumor was growing from main nerve in spine. When they opened lung the tumor was floating and fell in sergeant hand. They did not have to remove half of lung. I was prayed over by a prayer healer. What happened during surgery is what the healer told me , but did not tell the doctors. After that all right side from head on down I sweat and turn red,red. You would think I would have put a book on my face down the middle and got a sunburn. I am misable. I have to have cold, cold air conditioning and fans blowing on me and ice packs. Left side does not sweat or turn red. I have my left hand and arm that will get ice cold and right hand and arm normal. After surgery it was right hand by and arm then years later it switch over. 25 yrs now. Still the same . My Neologist said it was a nerve on my spine that they cut when they made a 13 inch incision. He gave a some kind of Syndrome. Heart Dr. Gave the same. I guess it is Harlequin. Been a long time. Good luck everyone

Diana says:

Hello, I find this very interesting as a few months ago I had 2.5. Kg schwannoma removed from the left side of my chest. It was sitting on top of my lung and I was very thankful to keep my lung. It was found incidentally through a scan because of some other issues. I had it removed. I woke from surgery with my children in the room looking strangely at me because my face was bright red but only on my right side with a line right down the centre of my face. The surgeon didn’t seem concerned or know what had caused it, he comented that I looked like a villen for the next bat man movie (I did look pretty strange). Anyway after recovering well from the surgery, I’m now trying to get fit again, I’ve started by walking and slow jogging and to my surprise whenever I exercise the right side of my face turns red and gets hot the other remains cold and white. Not ideal, but happy to have got through the surgery well. I had no idea what was going on so greatful to have found this article, because it is very strange.

Lisa Germany says:

Mine was noticed prior to being diagnosed with Lung Cancer in August of 2009. I’ve recovered, however, the cancer wrapped around my carotid nerve & sucked my left eye back in the socket so much, it looks droopy all the time. So, I have a white, dry droopy side & a super sweaty, flushed side to my face. It’s just as bad today as it was back then, though some people do regain normal function, depending on the injury to the nerves. I’m grateful to have recovered, even with the goofy, lopsided appearance.

Lola Jemifor says:

my husband was down with stroke that affected his right side and he has a drop foot, it also affected his memory and speech, he has been on medication and physiotherapy and getting better though slowly. we discovered he sweaths on the right side from the upper arm down the chest then the torso or upper thigh. The left side is always dry. This happens after eating hot food, excersise or when their is heat. that part of the body looks grey or dark and other times it is normal color. Although no pain but we have been so worried until I decided to check it out. Thanks for throwing some light on this issue.

Judi Mazziotti says:

I am a 72 yr. old very active woman, just recovering from my first bout ever of a month-long bronchitis. For three days I have noticed redness and heat on my left cheek and at the same time coolness and paleness on my right side. I didn’t notice a line if demarcation and did not pay attention to my sweating in the gym today, my 1st time back to exercising all month. Happy to have found this site. Wish some doctor would find a cure for those who suffer more than myself.

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