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Hypohidrosis is diminished sweating in response to appropriate stimuli. While hyperhidrosis is a socially troubling but benign condition, hypohidrosis can lead to hyperthermia, heat exhaustion, heat stroke and potentially death.^[1] An extreme case of hypohydrosis in which there is a complete absence of sweating and the skin is dry is termed anhidrosis.

Causes

Medications

Anticholinergic agents
Opioids
Botulinum toxin
Alpha-2 receptor antagonists
Clonidine
Barbiturates

Physical agents

Tumors
Burns
Radiation
Surgery
Scars
Sores

Dermatological

X-linked hypohidrotic ectodermal dysplasia
Incontinentia pigmenti
Bazex disease
Fabry disease
Miliaria
Sjogren syndrome
Systemic sclerosis
Graft-versus-host disease

Neuropathic

Multiple system atrophy
Dementia with Lewy bodies
Multiple sclerosis
Cerebrovascular accident
Tumour
Encephalitis
Cervical myelopathy
Diabetes mellitus
Guillain–Barre syndrome
Hereditary sensory and autonomic neuropathy
Alcoholism
Amyloidosis
Ross syndrome
Pure autonomic failure
Horner's syndrome

Diagnosis

Sweat is readily visualized by a topical indicator such as iodinated starch (minor test) or sodium alizarin sulphonate, both of which undergo a dramatic colour change when moistened by sweat. A thermoregulatory sweat test can evaluate the body’s response to a thermal stimulus by inducing sweating through a hot box ⁄ room, thermal blanket or exercise. Failure of the topical indicator to undergo a colour change during thermoregulatory sweat testing indicates hypohidrosis, and further tests may be required to localize the lesion.

Magnetic resonance imaging of the brain and ⁄ or spinal cord is the best modality for evaluation when the lesion is suspected to be localized to the central nervous system.

Skin biopsies are useful when anhidrosis occurs as part of a dermatological disorder. Biopsy results may reveal the sweat gland destruction, necrosis or fibrosis, in addition to the findings of the primary dermatological disorder.

Management

The treatment options for hypohidrosis and anhidrosis is limited. Those with hypohidrosis should avoid drugs that can aggravate the condition (see medication-causes). They should limit activities that raise the core body temperature and if exercises are to be performed, they should be supervised and be performed in a cool, sheltered and well-ventilated environment. In instances where the cause is known, treatment should be directed at the primary pathology. In autoimmune diseases, such as Sjogren syndrome and systemic sclerosis, treatment of the underlying disease using immunosuppressive drugs may lead to improvement in hypohidrosis. In neurological diseases, the primary pathology is often irreversible. In these instances, prevention of further neurological damage, such as good glycaemic control in diabetes, is the cornerstone of management. In acquired generalized anhidrosis, spontaneous remission may be observed in some cases. Numerous cases have been reported to respond effectively to systemic corticosteroids. Although an optimum dose and regime has not been established, pulse methylprednisolone (up to 1000 mg ⁄ day) has been reported to have good effect.

References

^ Chia, K. Y.; Tey, H. L. (2012). "Approach to hypohidrosis". Journal of the European Academy of Dermatology and Venereology 27 (7): 799–804. doi:10.1111/jdv.12014. PMID 23094789.

Notes

http://www.mayoclinic.com/health/anhidrosis/DS01050^{[full citation needed]}
MedlinePlus Encyclopedia Sweating - absent

UpToDate Contents

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1. ファブリー病の臨床的特徴および診断 clinical features and diagnosis of fabry disease
2. 遺伝性感覚自律神経ニューロパチー hereditary sensory and autonomic neuropathies
3. シェーグレン症候群の臨床症状：腺外型疾患 clinical manifestations of sjogrens syndrome extraglandular disease
4. 遺伝性皮膚症 the genodermatoses
5. サルコイドーシスの皮膚症状 cutaneous manifestations of sarcoidosis

English Journal

Patients with fabry disease after enzyme replacement therapy dose reduction versus treatment switch.

Weidemann F1, Krämer J, Duning T, Lenders M, Canaan-Kühl S, Krebs A, González HG, Sommer C, Uçeyler N, Niemann M, Störk S, Schelleckes M, Reiermann S, Stypmann J, Brand SM, Wanner C, Brand E.Author information 1Department of Medicine, Divisions of Cardiology and Nephrology, Comprehensive Heart Failure Center, Fabry Center for Interdisciplinary Therapy, and.AbstractBecause of the shortage of agalsidase-beta in 2009, many patients with Fabry disease were treated with lower doses or were switched to agalsidase-alfa. This observational study assessed end-organ damage and clinical symptoms during dose reduction or switch to agalsidase-alfa. A total of 105 adult patients with Fabry disease who had received agalsidase-beta (1.0 mg/kg body weight) for ≥1 year were nonrandomly assigned to continue this treatment regimen (regular-dose group, n=38), receive a reduced dose of 0.3-0.5 mg/kg (dose-reduction group, n=29), or switch to 0.2 mg/kg agalsidase-alfa (switch group) and were followed prospectively for 1 year. We assessed clinical events (death, myocardial infarction, severe arrhythmia, stroke, progression to ESRD); changes in cardiac, renal, and neurologic function; and Fabry-related symptoms (neuropathic pain, hypohidrosis, diarrhea, and disease severity scores). Organ function and Fabry-related symptoms remained stable in the regular-dose group. In contrast, estimated GFR decreased by about 3 ml/min per 1.73 m(2) (P=0.01) in the dose-reduction group, and the median albumin-to-creatinine ratio increased from 114 (0-606) mg/g to 216 (0-2062) mg/g (P=0.03) in the switch group. Furthermore, mean Mainz Severity Score Index scores and frequencies of pain attacks, chronic pain, gastrointestinal pain, and diarrhea increased significantly in the dose-reduction and switch groups. In conclusion, patients receiving regular agalsidase-beta dose had a stable disease course, but dose reduction led to worsening of renal function and symptoms. Switching to agalsidase-alfa is safe, but microalbuminuria may progress and Fabry-related symptoms may deteriorate.
Journal of the American Society of Nephrology : JASN.J Am Soc Nephrol.2014 Apr;25(4):837-49. doi: 10.1681/ASN.2013060585. Epub 2014 Feb 20.
Because of the shortage of agalsidase-beta in 2009, many patients with Fabry disease were treated with lower doses or were switched to agalsidase-alfa. This observational study assessed end-organ damage and clinical symptoms during dose reduction or switch to agalsidase-alfa. A total of 105 adult pa
PMID 24556354

Approach to hypohidrosis.

Verma P.
Journal of the European Academy of Dermatology and Venereology : JEADV.J Eur Acad Dermatol Venereol.2014 Mar 14. doi: 10.1111/jdv.12447. [Epub ahead of print]
PMID 24629071

Naegeli-franceschetti-jadassohn syndrome in a saudi arabian family.

Tubaigy SM1, Hassan HM.Author information 1Department of Medico Legal Medicine, Public Security, Ministry of Interior, P.O. Box 103223, Riyadh, 11695, Saudi Arabia.AbstractIn the course of applying to become a soldier, a 23-year-old Saudi Arabian man was found to have no fingerprints. Further medical examination has been carried out for the young man and for the rest of family members including two sisters, mother, and brothers except the father who had died sometime previously. Subsequent medical investigations suggested that he and his two brothers displayed most of the features of the Naegeli-Franceschetti-Jadassohn (NFJ) syndrome. These features included skin changes with hypo- and hyperpigmentation, hypohidrosis, dystrophy of the nails, diffuse thickening of the palms and feet, a lack of fingerprints (dermatoglyphics), and atrophic changes in the skin of the face; there were also dental anomalies. A typical feature of the Naegeli-Franceschetti-Jadassohn syndrome was found in a Saudi Arabian family. The aim of this study was to present this rare condition affecting a Saudi Arabian family and review the current literature on the subject.
Journal of forensic sciences.J Forensic Sci.2014 Mar;59(2):555-8. doi: 10.1111/1556-4029.12316. Epub 2013 Nov 21.
In the course of applying to become a soldier, a 23-year-old Saudi Arabian man was found to have no fingerprints. Further medical examination has been carried out for the young man and for the rest of family members including two sisters, mother, and brothers except the father who had died sometime
PMID 24261749

Japanese Journal

Horner's Syndrome with a Sensation of Warmth Due to Hypohidrosis

Higaki Manabu,Ishida Manabu,Nakamoto Yasuo,Saraya Takeshi
Internal Medicine 52(15), 1757-1758, 2013
NAID 130003365741

小児の症候性または潜因性局在関連性てんかんに対するトピラマートの有効性検証試験

大塚頌子
てんかん研究 31(1), 19-29, 2013
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NAID 130003362396