A localized form of aquagenic syringeal acrokeratoderma

A localized form of aquagenic syringeal acrokeratoderma
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水源性注射性肢端角皮病的局部形式

DOI:
10.1111/j.1365-2230.2009.03735.x
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发表时间:
2010
期刊:
Clincal and Experimental Dermatology
影响因子:
--
通讯作者:
A. Pembroke
A. Pembroke
中科院分区:
--
文献类型:
--
作者:
S. Flann;A. Pembroke

文献摘要

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水源性注射器角化病通常影响青少年或年轻成年妇女。它的特点是对称的,水肿,白色或半透明丘疹或斑块发展后,短暂暴露于水。手掌是更典型的表现部位,尽管脚底较少报道。我们报告一个不寻常的局部形式的这种情况发生在一个脚跟的内侧区域。一名21岁女性,右脚跟内侧有2年的红色斑块病史。在沐浴或淋浴后,它变得柔软,白色和更凸起,并在干燥后约1小时解决。病变无症状。其余的脚底和手掌未受影响,也没有相关的多汗症。体格检查中,右内侧足跟可见一个界限清晰的斑块,顶部有一层不透明的角质层,保留了正常的皮纹(图1a、c)。在桶水中浸泡5分钟后,斑块变得更白、不透明、更突出(图1b、d)。在皮肤活检的组织病理学检查中,可见明显的表面角化过度。诊断为水源性针剂性角化病的局部形式,并给予患者局部使用六水铝(Driclor; Stiefel, Maidenhead, Berkshire, UK),效果中等。“水桶里的手”标志是Yan等人创造的,描述了患者接受医学检查的方式,引起了只有当手浸入水中时才会出现的特征性皮肤病变,几乎可以被视为一种病症。这种病变通常出现在手掌上,但也有报道称这种情况发生在脚底,有一个病例报告影响了手背。病变可以是无症状的,如我们的病人,或伴随疼痛,刺痛或紧绷的感觉。发病机制尚不清楚,但已提出汗腺畸变和角质层紊乱。这可能是因为组织学上存在角化过度和扩张的内分泌管。在涉及手背的病例中,汗腺的腔有不规则的形状。
Aquagenic syringeal acrokeratoderma typically affects adolescent or young adult women. It is characterized by symmetrical, oedematous, white or translucent papules or plaques that develop after brief exposure to water. The palms are the more typical site for presentation, although the sole of the foot is less commonly reported. We report an unusual localized form of this condition occurring on the medial area of one heel. A 21-year-old woman presented with a 2-year history of a red patch on the right medial heel. It became soft, white and more raised after a bath or shower, and resolved about 1 h after drying. The lesion was asymptomatic. The rest of the soles and the palms were unaffected, and there was no associated hyperhidrosis. On physical examination, a well-demarcated plaque was seen on the right medial heel, with a more opaque keratinous layer on top with preservation of the normal dermatoglyphics (Figs 1a,c). After immersion in a bucket of water for 5 min, the plaque became whiter, opaque and more prominent (Figs 1b,d). On histopathological examination of a skin biopsy, prominent surface hyperkeratosis was seen. A diagnosis of a localized form of aquagenic syringeal acrokeratoderma was made and the patient was prescribed topical aluminium hexahydrate (Driclor ; Stiefel, Maidenhead, Berkshire, UK) with moderate success. The hand in the bucket sign was coined by Yan et al., and describes the manner in which patients present for medical examination, inducing the characteristic skin lesions that develop only when the hand is submerged in water, and it can almost be regarded as pathognomonic. The lesions typically present on the palms, but the condition has also been reported on the soles of the feet and there has been one case report affecting the dorsa of the hands. The lesions can be asymptomatic as with our patient, or be accompanied by pain, tingling or a sensation of tightness. The pathogenesis remains unclear but aberrations in the sweat glands and disorders of the stratum corneum have been suggested. This may be because histologically there is orthokeratotic hyperkeratosis and dilated eccrine ducts. In the case involving the dorsa of the hands, the lumina of the eccrine glands had an irregular shape.