Korean J Pediatr.  2016 Nov;59(Suppl 1):S125-S128. 10.3345/kjp.2016.59.11.S125.

A pediatric case of idiopathic Harlequin syndrome

Affiliations
  • 1Department of Pediatrics, Eulji University Hospital, Daejeon, Korea. kjh@eulji.ac.kr

Abstract

Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He had an unremarkable birth history and no significant medical history. Complete ophthalmological and neurological examinations were performed, and no other abnormalities were identified. Magnetic resonance imaging was performed to exclude lesions of the cerebrum and cervicothoracic spinal cord, and no abnormalities were noted. His final diagnosis was classic idiopathic harlequin syndrome. Herein, we report the first pediatric case of idiopathic harlequin syndrome in Korea.

Keyword

Harlequin syndrome; Autonomic dysfunction; Pediatrics

MeSH Terms

Cerebrum
Child
Diagnosis
Flushing
Hot Temperature
Humans
Korea
Magnetic Resonance Imaging
Male
Neurologic Examination
Pediatrics
Reproductive History
Spinal Cord
Sweat
Sweating
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