Harlequin syndrome in a young girl: An opportunity to rethink the terminology

Pediatr Dermatol. 2021 Jul;38(4):984-985. doi: 10.1111/pde.14681. Epub 2021 Jul 5.

Abstract

Harlequin syndrome is a rare disorder of the autonomic nervous system, presenting as unilateral reduced flushing and sweating of the face induced by exercise, stress, or heat. It is caused by a cervical sympathetic deficit located at the preganglionic or postganglionic level on the non-flushing side. We present a case of an 8 year old with harlequin syndrome and review the other dermatological conditions for which the term "harlequin" is part of the nomenclature.

Keywords: harlequin; harlequin phenomenon; harlequin sign; harlequin syndrome; terminology.

Publication types

  • Case Reports

MeSH terms

  • Autonomic Nervous System Diseases* / diagnosis
  • Child
  • Female
  • Flushing / diagnosis
  • Flushing / etiology
  • Humans
  • Hypohidrosis* / diagnosis
  • Sweating

Supplementary concepts

  • Harlequin syndrome