[Harlequin syndrome in a melanoderm patient]

Abstract

Introduction: Harlequin's syndrome is a neurological disorder due to a dysfunction of the sympathetic innervation of the face. It has been rarely reported in the literature. The authors report one case occurring in a melanoderm patient.

Observation: A 38-year-old melanoderm man, without any history of surgery or neck trauma, consulted for a strictly right unilateral facial hyperhidrosis. Clinical and radiological investigations concluded to an idiopathic Harlequin's syndrome. Therapeutic abstention was proposed because of non-invalidating symptoms.

Discussion: Harlequin's syndrome is a rare sudoral and vaso-motor disorder. On dark skin, flush and erythrosis may be subtle and the diagnosis less obvious. Etiologies are varied but essentially idiopathic. Its association with others dysautonomic facial syndromes is possible. Main differential diagnosis is the Frey's syndrome. Treatment is not clearly codified.

Keywords: Dysautonomia; Dysautonomie; Flushing; Hyperhidrose; Hyperhidrosis; Rougeur de la face; Sympathetic; Sympathique.