Harlequin syndrome R61.0

Lance et al. 1988

The term Harlequin syndrome (see also Harlequin discoloration, Harlequin ichthyosis) is used to describe, among other things, a syndrome in which the clinical aspect of a half-red and half-white face is strikingly prominent (Elboukhari Ket al. 2019).

Patients suffer from a hemifacial failure of sympathetic skin innervation with complete inability to vascular dilation and sweating on the affected "white" half of the face. Hyperemia and hyperhidrosis occur on the contralateral side (red half of the face) during exertion.

Hemiplegic loss of sympathetic skin innervation may occur postoperatively after spinal surgery (Heiler U et al. 2019). Harlequin syndrome can also occur as a paraneoplastic syndrome in association with Horner syndrome (e.g. in mediastinal ganglioneuroblastoma - Miyata K et al. 2021) .

Procedure depending on the cause. A causal therapy of hyperhidrosis is not possible. If disturbing, it can be treated with aluminium chloride solution. Partial blockade by botulinum injections can be tried.

1. Elboukhari Ket al. (2019) Idiopathic harlequin syndrome: a case report and literature review. Pan Afr Med J 33:141.
2. Heiler U et al (2019) Harlequin syndrome after scoliosis surgery. Orthopaed 48:1042-1044.
3. Heckmann M (2019) Erkrnakungen der Schweißdrüsen. In: Braun-Falco`s dermatology, venereology and allergology. Plewig G et al. Edit. Springer Verlag, Berlin pp. 1342-1343.
4. Miyata K et al (2021) Harlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome. Childs Nerv Syst 37: 2683-2686.