Harlequin Syndrome in Childhood
Keywords:
erythema, hyperhidrosis, harlequin syndrome.Abstract
Harlequin syndrome is a rare disorder of the autonomic nervous system, which consists of the sudden appearance of bouts of flushing and hemifacial diaphoresis that is usually due to heat, emotion or physical exertion. The absence of unilateral facial redness and sweating is due to ipsilateral dysfunction of the sympathetic cholinergic vasodilator and sudomotor pathway, at the central hypothalamic or spinal cord level from T1 to T4 (innervation of the face), preganglionic or postganglionic. Compensatory neuronal hyperactivity on the healthy side is thought to accentuate the cutaneous signs that identify this syndrome. A case of Harlequin syndrome is reported in a 4-year-old boy, diagnosed in Ciego de Ávila province. It is an infrequent entity in our setting, either due to underdiagnosis or the scarcity of cases reported in the scientific literature.
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