Article

Harlequin phenomenon is characterized by a strictly unilateral
erythrosis of the face with flushing and hyperhydrosis,
and controlaterally a
pale anhydrotic aspect.
This syndrome can occur alone or associated to other
dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome.
METHODS: We report three cases: two patients presented a Harlequin
sign, associated with Horner syndrome for one and Ross syndrome for the second.
The etiologic investigation was normal, allowing recognizing the idiopathic
nature of the disorder. For the third patient, Harlequin syndrome was observed in
a neoplastic context due to breast cancer, metastatic dissemination, and bone
metastases involving the right side of the T2 body. DISCUSSION: We reviewed the
literature: 108 cases have been described. This syndrome occurred alone in 48
patients and was associated with other dysautonomic syndromes such as Horner
syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six;
both Ross and Holmes Adie syndrome were associated five cases and associations
were not reported in five patients. The pathophysiological mechanisms of this
autonomic cranial neuropathy, the possible etiologies, and therapeutic management
were discussed. CONCLUSION: Harlequin phenomenon with flushing and unilateral
hyperhydrosis is rare, occurring alone or in combination with other autonomic
syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon
does not require specific treatment; sympathectomy may be discussed in the severe
cases with a significant social impact.
CI - Copyright (c) 2013 Elsevier Masson SAS. All rights reserved.
- Erythrose faciale

Langue : FRANCAIS