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对称性肢端角化病的临床、人口统计学和组织病理学特征。

Clinical, demographic and histopathological features of symmetrical acral keratoderma.

机构信息

Department of Dermatology, Nanfang Hospital, South Medical University, Guangzhou, China; Department of Dermatology, Dongguan Institute of Dermatology, Dongguan, China.

出版信息

Br J Dermatol. 2014 Apr;170(4):948-51. doi: 10.1111/bjd.12754.

Abstract

BACKGROUND

A series of cases of symmetrical acral keratoderma have been described recently in China. However, no studies about its demographic information and epidermal barrier function have been documented.

OBJECTIVES

To describe the clinical manifestation, demographic information and clinicopathological features of 71 cases with symmetrical acral keratoderma.

PATIENTS AND METHODS

Seventy-one cases with symmetrical acral keratoderma were retrospectively reviewed. Their demographic information, clinical manifestations, histopathology and epidermal barrier function were analysed.

RESULTS

Among these patients, there were 64 males and seven females, ranging in age from 4 to 53 years with an average age at onset of 27 ± 8·9 years. Clinical manifestation was characterized by brown hyperkeratotic patches over the dorsum of the hands, palms and feet, dorsal digits and wrists, elbows, knees and ankles. The lesions became dramatically whitish with mild swelling immediately after soaking in water and resolved spontaneously in winter. In patients, a moderate increase of transepidermal water loss (TEWL) from 16·16 ± 6·15 to 9·9 ± 4·21 g m(-2)  h(-1) (P = 0·0054) and a moderate decrease of skin hydration from 65·9 ± 5·06 to 42·58 ± 10·73 (P < 0·01) in comparison with the control group were observed. Histopathological examination revealed epidermal hyperkeratosis, acanthosis and papillomatous hyperplasia as well as dermal infiltration with a few lymphocytes.

CONCLUSIONS

Symmetrical acral keratoderma is characterized by symmetry, acra, keratinization and marked seasonal changes. The epidermal barrier function of the skin was negatively affected.

摘要

背景

最近中国描述了一系列对称性肢端角化病病例。然而,尚未有关于其人口统计学信息和表皮屏障功能的研究。

目的

描述 71 例对称性肢端角化病的临床表现、人口统计学信息和临床病理特征。

患者和方法

回顾性分析 71 例对称性肢端角化病患者。分析了他们的人口统计学信息、临床表现、组织病理学和表皮屏障功能。

结果

这些患者中,男性 64 例,女性 7 例,年龄 4 至 53 岁,平均发病年龄为 27 ± 8.9 岁。临床表现为手掌、足底、手背、手指背、腕部、肘部、膝部和踝部的褐色角化过度斑块。这些病变在浸水后立即变得明显苍白且轻度肿胀,冬季则自行缓解。与对照组相比,患者的经表皮水分丢失(TEWL)从 16.16 ± 6.15 g·m(-2)·h(-1)中度增加到 9.9 ± 4.21 g·m(-2)·h(-1)(P = 0.0054),皮肤水分从中度减少从 65.9 ± 5.06 g·m(-2)到 42.58 ± 10.73 g·m(-2)(P < 0.01)。组织病理学检查显示表皮角化过度、棘皮症和乳头瘤样增生以及真皮内有少量淋巴细胞浸润。

结论

对称性肢端角化病的特征是对称性、肢端、角化和明显的季节性变化。皮肤的表皮屏障功能受到负面影响。

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