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获得性特发性无汗症:一种常被漏诊的疾病。

Acquired idiopathic anhidrosis: a diagnosis often missed.

机构信息

Department of Dermatology, Changi General Hospital, Simei, Singapore.

National Skin Centre, Simei, Singapore.

出版信息

J Am Acad Dermatol. 2014 Sep;71(3):499-506. doi: 10.1016/j.jaad.2014.03.041. Epub 2014 May 22.

DOI:10.1016/j.jaad.2014.03.041
PMID:24856478
Abstract

BACKGROUND

Acquired idiopathic anhidrosis (AIA) is an uncommon entity characterized by anhidrosis in the absence of any neurologic or sweat gland abnormalities.

OBJECTIVE

The aim of this study was to characterize the clinical profile in a cohort of patients diagnosed with AIA at a tertiary dermatologic center.

METHODS

We retrospectively evaluated cases seen during a 10-year period. Inclusion criteria included all cases of generalized or partial anhidrosis with no obvious causes, confirmed by provocative starch-iodine sweat test. Ectodermal dysplasias, poral dysfunction from chronic dermatoses, autonomic dysfunction, and drug-induced causes were excluded.

RESULTS

Fifteen Chinese patients were diagnosed with AIA, mostly healthy young men with no significant medical or drug history, with variable extent of body surface area involvement. Serum immunoglobulin E, a complete blood cell count, thyroid function test, and antinuclear antibody levels were unremarkable. Anhidrotic areas revealed normal eccrine appendages with mild perivascular and perieccrine lymphocytic infiltrate. There were no neurologic abnormalities.

LIMITATIONS

This was a retrospective study.

CONCLUSION

Our study shows that AIA seems to be a heterogeneous group with no major dysfunction other than anhidrosis. Proper recognition and evaluation is paramount, especially for at-risk populations, so that appropriate measures on the prevention of heat injuries can be instituted.

摘要

背景

获得性特发性无汗症(AIA)是一种罕见的疾病,其特征为无汗,且无神经或汗腺异常。

目的

本研究旨在描述在一家三级皮肤科中心被诊断为 AIA 的患者队列的临床特征。

方法

我们对 10 年间的病例进行了回顾性评估。纳入标准为所有有明确病因的全身性或局部性无汗症,经淀粉-碘汗液激发试验证实。排除外胚层发育不良、慢性皮肤病引起的汗腺功能障碍、自主神经功能障碍和药物引起的原因。

结果

15 例中国人被诊断为 AIA,均为健康年轻男性,无明显的医疗或药物史,受累的体表面积大小不一。血清免疫球蛋白 E、全血细胞计数、甲状腺功能检查和抗核抗体水平均无异常。无汗区的外分泌腺正常,伴有轻度血管周围和外分泌腺周围淋巴细胞浸润。无神经异常。

局限性

这是一项回顾性研究。

结论

本研究表明,AIA 似乎是一组异质性疾病,除了无汗外,无其他主要功能障碍。适当的识别和评估至关重要,特别是对高危人群,以便采取适当的预防热损伤措施。

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Acquired idiopathic anhidrosis: a diagnosis often missed.获得性特发性无汗症:一种常被漏诊的疾病。
J Am Acad Dermatol. 2014 Sep;71(3):499-506. doi: 10.1016/j.jaad.2014.03.041. Epub 2014 May 22.
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Degranulation and shrinkage of dark cells in eccrine glands and elevated serum carcinoembryonic antigen in patients with acquired idiopathic generalized anhidrosis.获得性特发性全身无汗症患者的外分泌腺脱颗粒和暗细胞缩小及血清癌胚抗原升高。
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