Harlequin Syndrome

Excerpt

Harlequin syndrome is a rare dysautonomic syndrome first reported by lance et al. in 1988. It is characterized by hemifacial discoloration, sharply demarcated at the midline. It is caused by unilateral blockade of sympathetic fibers, which carry the vasodilator and sudomotor nerves to the face. It is primarily idiopathic, triggered by exercise, emotions, spicy food, or heat. However, it can also be a manifestation of underlying cervical and upper thoracic pathologies resulting in the compression of these sympathetic fibers or iatrogenic from the surgical or anesthetic procedures around the neck.

It is a benign self-resolutive syndrome. However, the sudden dramatic, colorful presentation of this condition can bring a lot of distress and anxiety to the patient and families. Familiarisation with this syndrome helps the clinicians identify the condition and appropriately direct patient care.