Aquagenic acrokeratoderma

Aquagenic acrokeratoderma
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DOI:
10.1111/j.1365-4632.2009.04304.x
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发表时间:
2010-05-01
影响因子:
3.6
通讯作者:
Wu, Liang-Cai
Wu, Liang-Cai
中科院分区:
医学4区
文献类型:
--
作者:
Luo, Di-Qing;Zhao, Yu-Kun;Wu, Liang-Cai

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背景水源性肢角皮病(AA)是一种罕见的疾病,女性好发,在短暂接触水后发生,干燥后几分钟至一小时消失。方法对4例中国AA患者进行报告和分析。结果男2例,女2例(年龄范围:14~33岁),有2周至10余年的手部白色小丘疹融合成水肿斑块病史;损伤在接触水5-10分钟内出现,在3-20分钟内开始消退,并在干燥后5分钟至1小时内消失。温水比冷水更快地引发病变。女性患者的病变可能是由清洁剂引发的。在另一名女性患者中,病变也涉及足部,并伴有掌跖红斑和多汗症。一名患者接触水后病变部位的活检显示角化过度、轻度颗粒增多和小汗腺管扩张。另一名患者病灶干燥后的活检显示表皮和真皮正常。 2例患者外用3%福尔马林酒精溶液治疗,2例患者外用3%硫酸铝钾溶液治疗,部分缓解,无任何不良反应。结论AA可发生于男性和女性,并可累及足部。温水比冷水更快地引发病变。外用 3% 福尔马林酒精和 3% 硫酸铝钾溶液可能是可选疗法。
BackgroundAquagenic acrokeratoderma (AA) is a rare condition with female predilection that occurs after brief water exposure and disappears minutes to an hour after drying. The pathogenesis remains unclear.MethodsFour Chinese patients with AA were reported and analyzed.ResultsThere were 2 males and 2 females (age range: 14-33 years) who presented with a 2-week to more than 10-year history of small white papules coalescing into edematous plaques on the hands; lesions appeared within 5-10 minutes of water exposure, began to regress in 3-20 minutes and disappeared within 5 minutes to one hour after drying. Warm water provoked the lesions more rapidly than cold water. Lesions of a female patient could be triggered by detergent. In another female patient, lesions also involved the feet and were associated with palmoplantar erythema and hyperhidrosis. Biopsy from the lesion of one patient after water exposure revealed hyperkeratosis, mild hypergranulosis, and dilatated eccrine ducts. Biopsy from the lesion of another patient after drying showed normal epidermis and dermis. Two patients were treated with topical formalin 3% in alcohol, and two with 3% potassium aluminium sulfate solution with partial relief without any adverse effects.ConclusionAA may occur in both males and females and may involve the feet. Warm water triggers the lesions more rapidly than cold. Topical formalin 3% in alcohol and 3% potassium aluminium sulfate solution may be optional therapy.