文献资料 > 汗孔角化症
Linear porokeratosis2014.12.29
线状汗孔角化症
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...
A case of porokeratosis plantaris palmaris et disseminata and literature review2014.10.08
合并播散性掌跖汗孔角化症病例报告及文献综述
合并播散性掌跖汗孔角化症(PPPD)是汗孔角化症中极其罕见的一种临床类型。本文报道了一例56岁的男性患者病例。其躯干、四肢无症状、褐色环状斑疹25年余,且皮损数目逐年渐增。近10年掌部出现类似皮疹。约一两年前,其跖部出现直径为2-3mm的疼痛性疣样的角化性点状丘疹,...
Identification of a locus (DSP2) for disseminated superficial porokeratosis at chromosome 12q21.2–24.212013.12.12
Summary Porokeratosis is a rare disorder of epidermal keratinization that is characterized by the presence of a border called the cornoid lamella. Disseminated superficial porokeratosis (DSP) is a subtype of porokeratosis, which is inherited as an au...
Fine mapping of the disseminated superficial porokeratosis locus to a 2.7 Mb region at 18p11.32013.12.12
Summary Disseminated superficial porokeratosis (DSP) is an autosomal dominant epidermal keratinization disorder. The genetic basis for DSP has not been clearly elucidated. We previously mapped the locus for DSP to a large region (5.7 Mb) at 18p11.3 ...
Reassessment of microarray expression data of porokeratosis by quantitative real-time polymerase chain reaction2013.12.12
Background: Porokeratosis (PK) is a heterogeneous group of keratinization disorders that exhibit similarities with psoriasis at both the clinical and molecular levels. Methods: The transcript levels of keratin (KRT) 6A, 16, 17, S100A7, A8, A9, p53 ...
Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin2013.12.12
ABSTRACT Porokeratosis is a disorder of keratinization showing a well-deÞ ned lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. We report familial (autosomal dominant with reduced penetrance) disseminated plaqu...
Different morphological variants of hypertrophic porokeratosis and disseminated lesions of porokeratosis of Mibelli: A rare co-existence2013.12.11
Abstract: Porokeratosis (PK) is a rare genodermatosis of abnormal epidermal keratinization. The etiology is unknown with varying presentation and an unpredictable outcome. Coexistence of the different variants of PK is seldom noted. Disseminated l...
Co-localization of lichen planus and porokeratosis of Mibelli2013.12.11
Abstract: Coexistence of lichen planus (LP) with a number of dermatological disorders is well documented. We are reporting here a case of co-localization of lichen planus with porokeratosis of Mibelli in a 40-year-old man who presented with some i...
The successful off-label use of photodynamic therapy for classic porokeratosis of Mibelli: case report2013.12.11
ABSTRACT: Porokeratosis of Mibelli is an uncommon chronic disorder of epidermal keratinization that should be treated because it can undergo malignant change into epithelial tumors on the lesions. At the moment, it represents a therapeutic challen...
Mibelli revisited: A case of type 2 segmental porokeratosis from 18932013.12.11
Abstract In autosomal dominant skin disorders, a pronounced mosaic involvement may sometimes be found to be superimposed on the ordinary nonsegmental lesions. Such ‘‘type 2 segmental manifestation’’ reflects loss of heterozygosity occurring at...
Duplication Including the EMILIN2 Gene in a Family with Porokeratosis of Mibelli2013.12.11
汗孔角化症家庭中EMILIN2基因的复制
汗孔角化症是一种罕见的表皮角质化疾病,以组织病理学上存在角质样板层为特征,由紧紧排列的角化不全细胞组成的一个细胞柱,其病因学目前仍不清楚。汗孔角化症是汗孔角化病的一个亚型,其临床表现为位于单侧四肢的单个斑块或少数大小不一的斑块。通常在幼年期发病,...
Hyperkeratotic variant of porokeratosis Mibelli with dermal amyloid deposits2013.12.11
伴有皮肤淀粉样蛋白沉积的角化过度型汗孔角化症
我们报道了一例伴有皮肤淀粉样蛋白沉积的角化过度型汗孔角化症病例。一名66岁的男性患者,在过去的几年中下肢有多个褐色角化性病变,左脚的第三脚趾有一个疣状结节,臀部有褐色疣状斑块。对右臀部区域的角化斑块进行病理组织学检查,结果显示为极度角化过度和角质样板层...
Porokeratosis: present concepts2013.12.10
AbstractPorokeratosis represents a group of disorders of epidermal keratinization which are characterized by the histopathological feature of the cornoid lamella, a column of tightly fitted parakeratotic cells. The aetiology of porokeratosis is still...
Genital porokeratosis: a series of 10 patients and review of the literature2013.12.10
Background:Porokeratosis (PK) is an uncommon disorder of epidermal keratinization with a unique clinical appearance, unknown aetiology, and an unpredictable outcome. Genital PK (defined as localized PK confined to the genital area in this study) is ...
Craniosynostosis, anal anomalies, and porokeratosis (CDAGS syndrome): Case report and literature review2013.12.10
A b s t r a c tCDAGS syndrome is an autosomal recessive syndrome characterized by craniosynostosis, large open fontanelles, hearing loss, anal anomalies, genitourinary malformations and porokeratosis. To our knowledge, only four families from differe...
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