Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net Letter
doi: 10.4103/ijdvl.IJDVL_767_16
PMID: 28799533

Unilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome

Crystal Zhen Yu Phuan1 , Hong Liang Tey2
1 National Skin Centre; Department of General Medicine, Yong Loo Lin School of Medicine, Singapore
2 National Skin Centre; Department of General Medicine, Lee Kong Chian School of Medicine, Singapore

Correspondence Address:
Hong Liang Tey
National Skin Centre, 1 Mandalay Road
Published: 09-Aug-2017
How to cite this article:
Yu Phuan CZ, Tey HL. Unilateral facial and upper truncal anhidrosis and absence of physiological flushing: A case of idiopathic harlequin syndrome. Indian J Dermatol Venereol Leprol 2017;83:740
Copyright: (C)2017 Indian Journal of Dermatology, Venereology, and Leprology


Unilateral absence of physiological facial heat-induced flushing and sweating is a very rare and unique presentation. These features characterize the Harlequin syndrome,[1] a rare autonomic disorder, which occasionally also involves the neck, upper thoracic region, and the arm.[1] It results from sympathetic deficits localized to the anhidrotic side, but can involve the parasympathetic neurons in the posterior and ciliary ganglia.[2]

A 13-year-old girl presented at the National Skin Centre, Singapore with unilateral anhidrosis and absence of heat-induced flushing over her left face, neck, and upper chest. Her symptoms started 5 years prior and had remained the same. Her medical history comprised a single afebrile seizure at 12 years of age; she was otherwise well. There were no birth complications or developmental delay. She was not on any medications and had no history of prior surgeries. There was no significant family history.

On examination, her vital signs were normal and there was no drop in postural blood pressure. A detailed neurological examination was unremarkable; specifically, there was no ptosis or miosis and pupils were equally responsive to light and accommodation; deep tendon reflexes were normal. She underwent a thermoregulatory sweat test using a method we have previously described.[3] After exercising in a temperature- and humidity-control room for 20 minutes, an absence of heat-induced vasodilatory response on her left face, neck, upper chest, and arm was observed. Minor starch-iodine test revealed associated unilateral well-demarcated anhidrosis in the same locations [Figure - 1].

Figure 1: Starch-iodine test after heat-induction revealed well-demarcated unilateral anhidrosis on the left side of the face, neck, chest and arm. There were normal amounts of sweating on the right side of the body and from left T4 dermatome downwards, which had turned the starch-iodine powder admixture purple

Her complete blood count, blood glucose, thyroid, liver, renal, and electrolyte panels were normal. Chest X-ray with apical lung views, brain magnetic resonance imaging, and electroencephalogram revealed no abnormalities. The patient was counselled on the benign nature of her condition, for which no medical treatment was required.

Harlequin syndrome has been increasingly accepted within the spectrum of partial dysautonomias, which affect the facial, and occasionally, brachial pupillary, sudomotor, and vasomotor responses. Physical signs of partial dysautonomias include the harlequin sign, generalized dysautonomia, Holmes–Adie pupils, and Ross and Horner Syndromes, which are found in more than 50% of harlequin syndrome patients.[4] Most cases of Harlequin syndrome in adults are primary in nature,[5] but the majority of childhood cases have been found to be secondary.[4] Such secondary causes of Harlequin syndrome [Table - 1][5] should be excluded before the condition is diagnosed to be primary in etiology. In dermatology literature, only three cases of Harlequin syndrome have been previously reported.

Table 1: Causes of secondary Harlequin syndrome

Based on neuroanatomy, the T1 preganglionic sympathetic neurons which innervate the eye were intact, as our patient had no oculomotor symptoms. Sympathetic outflow to the upper extremities and trunk, innervated by the T4 and 5 preganglionic sympathetic neurons, were intact, as sweating was normal, below our patient's chest. Theoretically, harlequin syndrome in our patient may be caused by the occlusion of the anterior radicular artery, which solely supplies the second and third spinal thoracic segment and does not significantly contribute to the overall spinal cord blood flow.[1] However, one limitation of this study is that the identification of this possible abnormalityin vivo will be difficult, as the vessel and the area involved is very small.

Sympathetic cutaneous adrenergic vasoconstrictor nerves are known to co-locate with sympathetic cutaneous vasodilator nerves, and the latter are activated during hyperthermia.[2] Our patient had idiopathic disruption of her left sympathetic fibres, which can result in an increased expression of alpha-adrenoreceptors in denervated vessels. This can sensitize the cutaneous vessels to circulating catecholamines and result in increased vasoconstriction.[2]

With regards to the management of primary Harlequin syndrome, patients should be educated regarding the benign nature of their condition, which requires no medical treatment. Several techniques such as contralateral sympathectomy, botulinum toxin injections, stellate ganglion block, and costotransversectomy for symptomatic relief have been attempted in those who experience debilitating social embarrassment with variable results.[5]

Patients with anhidrosis or cutaneous vascular response abnormalities typically consult dermatologists. We presented a rare and unique presentation of unilateral anhidrosis and absence of physiologic vasodilation on the face due to primary Harlequin syndrome. Dermatologists' knowledge of this syndrome will enable expedient diagnosis and screening of secondary causes in patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: The sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988;51:635-42.
[Google Scholar]
Charkoudian N. Skin blood flow in adult human thermoregulation: How it works, when it does not, and why. Mayo Clin Proc 2003;78:603-12.
[Google Scholar]
Lim JH, Choo W, Chang JH, Tey HL, Chong WS. Application of iodinated starch powder using an atomizer spray gun-A new and effective tool to evaluate hypohidrosis. Skin Res Technol 2016;22:370-4.
[Google Scholar]
Bremner F, Smith S. Pupillographic findings in 39 consecutive cases of Harlequin syndrome. J Neuroophthalmol 2008;28:171-7.
[Google Scholar]
Willaert WI, Scheltinga MR, Steenhuisen SF, Hiel JA. Harlequin syndrome: Two new cases and a management proposal. Acta Neurol Belg 2009;109:214-20.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections