文献简介

出版社:Dermatology Online Journal

作  者:SK Malhotra, KJPS Puri, Tanu Goyal, KS Chahal

编  号:

关键字:Linear porokeratosis

年  份:2007   点击量:838

文献摘要 全文翻译

Abstract
Linear porokeratosis is a rare variant. It can be present at birth or can develop in adult life. Lesions of linear porokeratosis are grouped and linearly arranged along the lines of Blaschko. On the extremities it affects the distal portion more than the proximal areas. On the trunk these can be zosteriform in distribution. Lesions of linear porokeratosis probably result from an abnormal clone of epidermal precursors. A 20 year old male presented with annular plaques in linear pattern following the lines of Blaschko over the left upper limb extending up to the axilla present since childhood. The lesions had atrophic centre and raised hyperkeratotic borders. The lesions were more proximal than distal. Few scattered lesions were present on left side of trunk. There was no family history of such lesions. Systemic examination of patient was normal. On histopathological examination there was hyperkeratosis and parakeratosis. A coronoid lamella was present. At the base of coronoid lamella thinned out granular layer and necrotic keratinocytes were also seen. In the dermis pigment incontinence and perivascular lymphocytic infiltrate were present. This case is being reported because of its rarity. It is an atypical presentation because the lesions were disposed more over proximal than distal area of upper limb. Linear porokeratosis is associated with an increased risk of malignant transformation.
摘要
线状汗孔角化症是一种罕见的临床疾病,多发生于出生时或成年期,其皮损成簇状分布,并沿Blaschko线呈线性排列。四肢皮损通常累及远端,躯干皮损呈带状分布。皮损可能是由于表皮前体细胞异常增殖所致。一位20岁男性患者自儿童期始,其左上肢可见沿Blaschko线线性分布的环状斑块,中央萎缩,边缘角化隆起,并延伸至腋下。皮损多集中于上肢近端,躯干左侧可见少量散在性皮损。患者否认类似疾病家族史,系统检查无异常。组织病理学检查显示角化过度和角化不全,见鸡眼样板层,其下颗粒层细胞缺失,角化细胞坏死。真皮层内色素失禁,血管周围淋巴细胞浸润。本系病例罕见,具有非典型的临床特征,因与上肢远端相比,皮损更集中分布于上肢近端。线性汗孔角化病伴有较高的恶变风险。