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 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
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
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter To Editor
2008:74:4;404-405
doi: 10.4103/0378-6323.42927
PMID: 18797081

Idiopathic generalized anhidrosis: A feature of panautonomic failure

Sudip Kumar Ghosh1 , Debabrata Bandyopadhyay1 , Biman Kanti Roy2 , Susmit Haldar3 , Gobinda Chatterjee1
1 Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, India
2 Department of Neuromedicine, R.G. Kar Medical College, Kolkata, India
3 Calcutta Skin Institute, Kolkata, India

Correspondence Address:
Sudip Kumar Ghosh
Vill+P.O- Rajballavpur (Via -Maslandpur), Dist-24 Parganas (N), West Bengal - 743 289
India
How to cite this article:
Ghosh SK, Bandyopadhyay D, Roy BK, Haldar S, Chatterjee G. Idiopathic generalized anhidrosis: A feature of panautonomic failure. Indian J Dermatol Venereol Leprol 2008;74:404-405
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Anhidrosis refers to the absence of sweating in the presence of appropriate stimuli. Anhidrosis may result from abnormalities of sweat glands or from autonomic dysfunction. [1] It can be both congenital or acquired, and can be generalized or localized in nature. Chronic idiopathic anhidrosis has also been described as a forme fruste of acute panautonomic neuropathy. [2] We are reporting here a case of panautonomic dysfunction in a patient who presented with idiopathic generalized anhidrosis.

A 40 year-old woman presented with a history of recurrent bouts of high fever for the preceding 15 years. There was a generalized lack of sweating since childhood, except over the left half of her face, which also eventually became anhidrotic for the last 15 years. On presentation, she had a total absence of sweating, heat intolerance, as well as fecal and urinary urgency. Cutaneous examination showed shiny, smooth, hairless, dry skin. No sweating was visible even on provocation (with vigorous exercise and by taking hot drinks) and the result of the iodine-starch test was negative. Systemic examination revealed that the right pupil was irregularly dilated, the light reflex was absent in both the eyes although the accommodation reflex was intact; the extraocular muscle function was normal. In addition, she had postural hypotension. There was evidence of cardiac dysautonomia in the form of a decrease in heart rate variability with deep breathing and an abnormal Valsalva response. Higher mental functions, muscle tone and power, as well as both superficial and deep reflexes were normal. Routine investigations (including blood sugar), ultrasonography of the abdomen, electrocardiogram, magnetic resonance imaging of the brain, and nerve conduction velocity studies of the peripheral nerves did not reveal any abnormalities. Cutaneous histopathology was normal with preservation of the sweat glands.

A large number of cutaneous or systemic conditions may underlie anhidrosis. These include neurologic disorders such as Guillain-Barre syndrome, heatstroke, diabetes, congenital disorders including ectodermal dysplasia, drugs, autonomic neuropathy, infections of or trauma to the sweat glands, burns, and excessive dehydration.

Localized anhidrosis is of limited clinical importance apart from its diagnostic value in leprosy. On the other hand, generalized or extensive anhidrosis (such as in the case of anhidrotic ectodermal dysplasia) may lead to hyperpyrexia with its associated complications. [1] Disorders of the autonomic nervous system may result from pathology of either the central or peripheral nervous systems. [3] Pure pandysautonomia is clinically characterized by some combinations of anhidrosis, orthostatic hypotension, paralysis of papillary reflexes, loss of lacrimation and salivation, impotence, impaired bladder and bowel function, flushing, and heat intolerance. Somatosensory and reflex functions are usually spared. [4] This patient showed an absence of sweating along with other features of autonomic failure in the form of urinary and fecal urgency, postural hypotension, asymmetry of the pupils with an absence of a light reflex, as well as an abnormal Valsalva response. Moreover, a histopathological examination of the skin did not reveal any abnormalities, indicating that the anhidrosis was due to autonomic failure. The neuroimaging and nerve conduction velocity were normal; hence, a diagnosis of idiopathic generalized anhidrosis was made.

Patients with generalized anhidrosis have a dangerous inability to tolerate heat. Therefore, when the weather is dry and hot, the inability to sweat can be life-threatening due to the potential to develop heat stroke. Anhidrosis may go unrecognized until a substantial amount of heat or exertion fails to cause sweating. This forms the background of the hyperpyrexia seen in our patient. In rare cases, family members of such patients may have anhidrosis and anisocoria, [5] but our patient did not have any such family history. In conclusion, anhidrosis can be present in some stages of autonomic failure, and a dermatologist must be aware of this fact so as to be able to routinely rule out the possibility of autonomic dysfunction when dealing with a case of anhidrosis.

References
1.
Coulson IH. Disorders of sweat glands. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's text book of dermatology.7 th ed. Oxford: Blackwell Science; 2004. p. 45.1-23.
th ed. Oxford: Blackwell Science; 2004. p. 45.1-23. '>[Google Scholar]
2.
Goldsmith LA. Disorders of eccrine sweat glands. In: Freedberg IM, Eisen AZ, Wolff K, Frank Austen K, Goldsmith LA, Katz SI, editors. Fitzpatrick's dermatology in general medicine. 6 th ed. New York: McGraw-Hill; 2003. p. 699-707.
th ed. New York: McGraw-Hill; 2003. p. 699-707. '>[Google Scholar]
3.
Low PA, Engstrom JW. Disorders of autonomic nervous system. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine. 16 th ed. New York: McGraw-Hill; 2005. p. 2428-34.
th ed. New York: McGraw-Hill; 2005. p. 2428-34.'>[Google Scholar]
4.
Disorders of the Autonomic Nervous System, Respiration and Swallowing. In: Ropper AH, Brown RH eds. Adam's and Victor's Principles Of Neurology.8 th ed. New York: McGraw-Hill; 2005. p. 452-79.
th ed. New York: McGraw-Hill; 2005. p. 452-79.'>[Google Scholar]
5.
RadhaKrishnan K, Anand IS, Sahasranam KV, Madhusudhanan M, Banerjee AK, Chopra JS, et al . Anhidrosis with anisocoria: Clinical and autonomic evaluation. Clin Neurol Neurosurg 1983;85:31-9.
[Google Scholar]

Fulltext Views
71

PDF downloads
36
Show Sections