Harlequin Syndrome

Sudden onset of asymmetrical flushing on left sign of face with increased sweating. Seems to be exercise induced following walk/run but also flairs up when working in yard on hot day. Right side of face remains normal color and does not sweat.

Initial internist exam/tests are normal other than long time misalignment in c-spine vertebrae. Soon to have MRI of c-spine and upper t-spine vertebrae.

My personal online research took me to Harlequin Syndrome but no blog poster provided doctor or medical research facility information where they had been screened/treated.

I'm looking for a doctor with some experience or at least knowledge of symptoms and possible causes to investigate and eliminate before deciding it is a benign condition I have to live with. Neurologist? Orthopedic? Vascular? Location is not important.

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I experience assymmetrical flushing at times, other times it symmetrical. I have a periodic fever syndrome called TRAPS. My main symptoms are fever and muscle and joint pain, but i have rashes that seem to be brought on by exercise, heat, stress, nerves, etc. I went misdiagnosed for several years and was finally diagnosed at the Mayo Clinic. Hope this helps.

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Thank you Angela. At this point I only have the asymmetrical flushing from my upper lip upward through my forward, complete with non-sweating on the non-flushed side. No fever. I already take as needed Nabumetone for some joint swelling and pain so I'm not sure if I have muscle aches, etc. Good to know that Mayo helped you and I'll be researching more. My next step is a spinal MRI to show doctors that I need a symptom management plan.

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Hi there i am Desiree (50) i have had these symptoms since last year, and my Mdjust blew it off, then I got a new Md and wow she was on the ball! I saw a neuro and endochronologist and they came wup with this diagnosis, I was told could be from maybe a growth on my Thyroidm, after some testing they did find a nodule on the Thyroid which presses down on the nerves,
I am just glad that there is a name to what was Happening, I brought pictures in to my doc, so she can see first hand on how I looked,

sometimes surgery can relieve the symptoms if they are real bad.
So maybe the next time you see your md mentuion about the thyroid, I also got an excellent article on Harlequin from Web Md.

Wish you all the very best, keep me posted

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Thank you for the thyroid clue. I have a history of hyperthyroidism with two remissions following medications treatment only. I do not take any meds now over 20 years later and still have an intact, functioning thyroid. Glad I refused permanent "death" of my thyroid decades ago.

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Hello all,

I have similar experiences as Desiree.

I have Harlequin Syndrome since June 2009. And experiencing lots of health-problems since than.... (from one day to the other).
I was already thyroid-patient (Hashimoto's) but everything seemed OK.
Even last year nothing could be found (blood and X-thorax were OK). At that time I felt that my neck had grown on one side (the 'sweating' side).
After starting a group at Facebook I heard that normal bloodvalues don't mean anything. That might explain my health-problems (which seem to be related to thyroid-disorder). And after lowering the dosage of my thyroid medication I start feeling the neck again. But this time it becomes worse than last year. I will visit the endocrinologist again tomorrow.

It is known that there are more cases of people with Harlequin which is caused by s nodule pressing down on the nerves.
According to those cases, the damage to the nervous system can not be 'restored'. But perhaps it can take away some of the health-problems I experience.

There is a website about this Syndrome and some related Syndromes: http://www.ross-syndrome.com

There is also a group at Facebook about these Syndromes: http://www.facebook.com/groups/ross.and.related.syndromes/

In this group (privacy: 'closed') it is possible to exchange experiences with other patients....

Hope to see you all there...

Best regards,


P.s. @ Desiree: I really like to know how your story has developed in this last month.....

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Hi Sip,
I asked to join the FB group. Peculiar phrase though - "add a comment to the Syndrome which you are diagnosed with" - how do I get more than a self-diagnosis? Neurologist appt not until Jan. 31, 2012. Should I search for an endocrinologist with knowledge about thyroid and harlequin connections? Seems like a good path to follow.

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Hi Bray (and others),

This link might be interesting to you and others with Harlequin. It seems there are more ways in which Harlequin might be 'caused'.

This link is about a thyroid-disorder (not just goiter, but also thyroid artery...):

I'm expecting at least two researches now:
1. a scan of the neck-area to determine what could explain the growth of my neck (unilateral)
2. a research for the thyroid artery.

Next tuesday I will visit the neurologist. I will ask her for a PET-scan of the neck. With a PET-scan can be determined which part of the sympathetic nervous system is still correctly working. I have the feeling that not just the sweating-glands have been 'damaged' but also some other glands (including one side of the thyroid?). A hope a PET-scan will show that I might be right. That might also explain why the other side of the thyroid seems to grow (compensatory growth?).....


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