Harlequin syndrome, although its symptoms can be seen on the skin, is not a dermatological disease at all. Harlequin syndrome is a rare neurological disorder. It is caused by disturbances in the work of the facial nerve fibers. As a result, half of the face is pale and the other half red. But this symptom can also be a sign of thyroid disease and diabetes complications. What exactly causes Harlequin syndrome and how is it treated?

Harlequin syndrome(idiopathic Harlequin syndrome) was first described in the medical literature in … 1988, based on the observation of 5 patients.

It turned out that after intense physical exertion, the faces of these patients resembled the half-masked (or makeup) face of the hero of the dell'arte comedy - Harlequin. With the difference that in the observed patients: one half of the face was red, the other white, and separated by a clear, vertical (!) Demarcation line.

Where did this strange Harlequin syndrome come from and what diseases does it herald?

Harlequin syndrome: symptoms

Redness and sweating after exercise, heat or emotion are a natural reaction of our nervous system. After all, it is he who controls our breathing, sweat glands and blood vessels.

It turns out, however, that in some people this mechanism is disturbed, and this disorder causes them to sweat and turn red only on one side of the body.

This disorder concerns the activity of sympathetic fibers innervating the face. In response to exercise, warmth and emotions, the "sick" half of the face remains pale and dry, and the he althy half of the face turns red, sweats (often profusely) and its blood vessels widen considerably. The two halves are clearly separated from each other, as if someone had drawn a straight line between them.

It also happens rarely that nerve damage also affects one hand or one side of the chest.

Harlequin syndrome: causes

All the causes of Harlequin syndrome are not yet fully understood. The ones that have been observed by researchers of this disease so far can be divided into 2 groups.

The first one includes diseases such as:

  • goiter of the thyroid gland
  • thyroid cancer
  • brain-stem infarction
  • syringomyeliacord
  • Pancoast tumor
  • upper mediastinum tumors
  • obstruction of root arteries
  • HSV 1 infection - herpes or cytomegalovirus

The second group of Harlequin syndrome causes include … surgical procedures, such as:

  • thyroid removal surgery
  • operations on the chest
  • insertion of the central needle
  • anesthesia of the alveolar nerves during dental procedures

Harlequin syndrome: a symptom of other diseases

It also turns out that Harlequin syndrome can also be a symptom of other diseases. So pay attention to how your faces react, because redness of only one half of it can herald:

  • diabetic neuropathy
  • amyotrophic lateral sclerosis
  • Guillain-Barré syndrome

Harlequin syndrome: treatment

Harlequin syndrome is more common in women than in men. In order to accurately diagnose its causes, the patient undergoes extensive neurological and ophthalmological examinations. Although usually a patient with symptoms of Harlequin syndrome first reports to … a dermatologist.

The course of Harlequin's syndrome is mild. Sometimes the so-called Adi's syndrome, i.e. a slow reaction of the pupils to light.

There are currently no treatments for Harlequin syndrome. Patients with this disease causing great psychological discomfort are offered the so-called unilateral sympathectomy. It is a procedure that destroys the nerves in the sympathetic nervous system on the "sick" side of the face, but it is fraught with complications.

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