Orphanet: Harlequin syndrome

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Harlequin syndrome

Disease definition

Harlequin syndrome (HSD) is an autonomic disorder occurring at any age and characterized by unilateral flushing and sweating, involving the face and sometimes arm and chest, in condition of thermal, exercise or emotional stress without sympathetic ocular manifestations. However, tonic pupils, parasympathetic oculomotor lesion and pre- or postganglionic sudomotor sympathetic deficit can rarely occur.


Classification level: Disorder
  • Synonym(s):
    • Progressive isolated segmental anhidrosis
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: G90.8
  • OMIM: -
  • UMLS: C2029348
  • MeSH: C535634
  • GARD: 8610
  • MedDRA: -
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