Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
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
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
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 - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
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
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
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
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Case Letter
2017:83:5;596-598
doi: 10.4103/ijdvl.IJDVL_541_16
PMID: 28749384

Partial dysautonomia: An interesting presentation

Subuhi Kaul1 , Chander Grover1 , Gopal K Das2
1 Department of Dermatology and STD, University College of Medical Sciences, Guru Teg Bahadur Hospital, New Delhi, India
2 Department of Ophthalmology, University College of Medical Sciences, Guru Teg Bahadur Hospital, New Delhi, India

Correspondence Address:
Chander Grover
Department of Dermatology and STD, University College of Medical Sciences and GTB Hospital, New Delhi - 110 095
India
Published: 26-Jul-2017
How to cite this article:
Kaul S, Grover C, Das GK. Partial dysautonomia: An interesting presentation. Indian J Dermatol Venereol Leprol 2017;83:596-598
Copyright: (C)2017 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

We report an interesting case with unilateral flushing and hyperhidrosis diagnosed to have an uncommon presentation of harlequin sign with Holmes–Adie syndrome.

A 45-year-old lady presented with a 15-year history of profuse sweating from the right half of her scalp, face, and neck with episodes of ipsilateral flushing associated with an uncomfortable sensation of warmth on the contralateral side. The intensity of sweating had progressed over the years to unbearable proportions. Simultaneously, she developed pain at the back of neck with restricted motion, along with episodic right-sided headache and difficulty in fixing gaze for prolonged periods. There was no history of trauma or any significant illness.

Examination at rest was unremarkable. However, post heat exposure or exertion, a distinct erythema and hyperhidrosis confined to right side of face, neck, and scalp with a sharp midline demarcation, was seen [Figure - 1]. Starch-iodine testing demonstrated anhidrosis on the left forehead [Figure - 2]. Ophthalmological examination revealed the presence of anisocoria [Figure - 3] with a right sided, irregularly dilated, tonic pupil (sluggish response to light or accommodation as compared to the normal left pupil). Upon instilling pilocarpine (0.1%) drops, the tonic pupil was seen to constrict much more than the normal left pupil. Neurological evaluation revealed bilateral hyporeflexic biceps and supinator tendon responses and absence of triceps, knee, and ankle deep tendon reflexes with a normal plantar response. Power for all major muscles groups was normal. Magnetic resonance imaging (MRI) of the cervical spine showed a diffuse disc bulge at C3/C4 level causing indentation of thecal sac and effacement of bilateral exiting nerve roots. Blood investigations including folate levels, rapid plasma reagin (RPR) test and autoimmune profile were normal.

Figure 1: Right sided flushing and hyperhidrosis as noticed on the face
Figure 2: Starch iodine test demonstrating anhidrosis on the left half of forehead
Figure 3: Right sided pupil showing irregular outline. It is also larger (tonically dilated) as compared to left pupil

Considering her presentation, she was diagnosed as partial dysautonomia in the form of harlequin sign with Holmes-Adie syndrome. She was explained the benign nature of her condition, advised to avoid extreme temperature and prescribed topical aluminium chlorohydrate 20% lotion at night during summer. The patient has remained comfortable for the past year.

Partial dysautonomia is characterized by abnormal regulation of the autonomic nervous system, resulting in symptoms like focal or generalized decrease in sweating, flushing, orthostatic hypotension, aberrant ocular responses and altered cardiovascular reflexes occurring either as isolated primary or secondary phenomenon, or as a combination.[1] It is an uncommon condition, which although essentially benign, can be extremely debilitating.

Among the major partial dysautonomic syndromes are the harlequin syndrome, described by Lance et al. in 1988. It is a rare disorder of sympathetic dysfunction characterized by unilateral hypohidrosis with contralateral flushing of face in response to physical exertion or heat.[2] It can occur as an isolated anomaly, or in association with other dysautonomic syndromes. A recent review of 108 cases found that 44.4% cases had harlequin syndrome alone; while 35.1% cases presented with Horner's syndrome. The association with Holmes-Adie syndrome, as seen in our case, is distinctly uncommon (5.5% cases).[3] Known causes of acquired disease include space occupying lesions like mediastinal neurinoma, thoracic neurofibroma, cervical syrinx, intramedullary astrocytoma, along with iatrogenic causes viz. jugular vein catheterization, paravertebral thoracic blocks, thoracic sympathectomy, etc.[4]

Ross syndrome and Holmes–Adie syndrome are rarer syndromes whose salient features are presented in [Table - 1].

Table 1: Comparative features of partial dysautonomic syndromes

In these cases, the pathology lies on the anhidrotic side and is due to a disruption of the sympathetic nervous system. The result is compensatory flushing and hyperhidrosis contralaterally.[5] The constellation of signs and symptoms offer a clue to the level of sympathetic damage. A combination of harlequin sign with Horner's syndrome points towards a lesion of the superior cervical ganglion, whereas harlequin sign with Ross or Holmes-Adie syndrome implicates an anomalous postganglionic cholinergic parasympathetic and sympathetic system response (supplying the iris smooth muscles and the eccrine glands).[5] This also leads to decreased ipsilateral cutaneous blood flow. Any vasodilatory signal thus result in ipsilateral pallor due to the vasoconstriction, giving a flushed appearance on the contralateral side with a definite midline demarcation.

Hyperhidrosis can be a very distressing complaint, as in our patient. Various management options have been described for this. If general measures of avoiding warm ambient temperatures and excessive exertion are deemed insufficient or not practical by the patient, they could be prescribed topical antiperspirant preparations. In severe cases, botulinum toxin may also be advised. Surgical sympathectomy is reserved for severe cases, unresponsive to above measures. Our patient found the use of topical antiperspirant lotion acceptable and relieving.

This case serves to sensitize dermatologists to this uncommon association. A comprehensive neurological and ophthalmological investigation is warranted along with a need to rule out malignancies in late onset cases. For idiopathic disease, counseling may prove sufficient but treatment modalities should be discussed and offered, if the need arises.

Declaration of patient consent

The patient has given her Verbal consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Bacon PJ, Smith SE. Cardiovascular and sweating dysfunction in patients with Holmes-Adie syndrome. J NeurolNeurosurg Psychiatry 1993;56:1096-102.
[Google Scholar]
2.
Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: The sudden onset of unilateral flushing and sweating. J NeurolNeurosurg Psychiatry 1988;51:635-42.
[Google Scholar]
3.
Guilloton L, Demarquay G, Quesnel L, De Charry F, Drouet A, Zagnoli F. Dysautonomic syndrome of the face with harlequin sign and syndrome: Three new cases and a review of the literature. Rev Neurol (Paris) 2013;169:884-91.
[Google Scholar]
4.
Willaert WI, Scheltinga MR, Steenhuisen SF, Hiel JA. Harlequin syndrome: Two new cases and a management proposal. ActaNeurolBelg 2009;109:214-20.
[Google Scholar]
5.
Kaur S, Aggarwal P, Jindal N, Dayal S, Jairath V, Jain VK, et al. Harlequin syndrome: A mask of rare dysautonomic syndromes. Dermatol Online J 2015;21. pii: 13030/qt3q39d7mz.
[Google Scholar]

Fulltext Views
538

PDF downloads
161
Show Sections