Harlequin syndrome: the sudden onset of unilateral flushing and sweating.

Abstract

SUMMARY Facial flushing and sweating were investigated in five patients who complained of the sudden onset of unilateral facial flushing in hot weather or when exercising vigorously. One patient probably suffered a brainstem infarct at the time that the unilateral flush was first noticed, and was left with a subtle Homer's syndrome on the side opposite to the flush. The other four had no other neurological symptoms and no ocular signs of Homer's syndrome. Thermal and emotional flushing and sweating were found to be impaired on the non-flushing side of the forehead in all five patients whereas gustatory sweating and flushing were increased on that side in four of the five patients, a combination of signs indicating a deficit of the second sympathetic neuron at the level of the third thoracic segment. CT and MRI of this area failed to disclose a structural lesion but latency from stimulation of the motor cortex and thoracic spinal cord to the third intercostal muscle was delayed on the non-flushing side in one patient. The complaint of unilateral flushing and sweating was abolished in one patient by ipsilateral stellate ganglionectomy. The unilateral facial flushing and sweating induced by heat in all five patients was thus a normal or excessive response by an intact sympathetic pathway, the other side failing to respond because of a sympathetic deficit. The onset in the four cases of peripheral origin followed strenuous exertion, which suggested that an anterior radicular artery may have become occluded at the third thoracic segment during torsion of the thoracic spine.One of the most mysterious, dramatic and colourful of autonomic syndromes is the sudden appearance of flushing limited to one side of the face, a symptom that does not appear to have received attention in the past. The descriptive title of "harlequin syndrome" comes to mind although the analogy has been drawn previously by paediatricians who apply the term "harlequin colour change" to vasomotor instability in the newborn causing flushing of the dependent half of the body.1Unilateral sweating in response to spicy foods, heat and exercise may develop spontaneously as "idiopathic hyperhidrosis" which is sometimes accompanied by flushing2 3 and sometimes not.4 5 Unilateral flushing as a primary complaint does not seem to have been previously recorded.The stimulus for the present study was provided by five patients who observed asymmetry of facial flushing in hot weather or after exertion. Our investigations were planned to determine which side was at fault and to elucidate the mechanism of the syndrome. Case reportsThe main clinical characteristics are summarised in

Hemifasiyal Atrofinin Eşlik Ettiği Harlequin Sendromu

“…Blefaropitozis, miyozis, fasiyal anhidrozis ve iris hipokromisi klinik olarak Horner sendromu olarak tanımlanmaktadır 4,5 …”

Harlequin Syndrome as a Complication of Epidural Anesthesia

“…First described by Lance et al, 8 harlequin syndrome is characterized by unilateral flushing of the face with a sharp demarcation down the midline. The flushing may also be accompanied by sweating and is usually elicited by hot temperatures, emotional response, or strenuous exercise.…”

“…After potentially life-threatening causes have been excluded, other conditions that should be considered include idiopathic hyperhidrosis, 1,3 Frey syndrome, 4,5 Horner syndrome, 6 brain or thoracic tumors, 1,7 and harlequin syndrome. 8 Differential diagnoses for unilateral facial flushing with distinguishing features are shown in the Table.…”

Harlequin Syndrome

“…Lance et al first described and named it based on resemblance to colorful harlequin masks [1]. This analogy is also used to describe a severe form of congenital ichtathyosis and these entities must not be confused.…”

“…It is characterized by asymmetric flushing, neck and sometimes upper thoracic region in response to heat, exercise or emotional factors [1].…”