慢性苔藓样角化病的临床及组织病理特征

慢性苔藓样角化病临床罕见,属良性、慢性、进行性苔藓样角化性皮肤病。其病因和发病机制不明。临床典型表现为四肢、臀部无症状性、对称性并呈线状或网状分布的紫红色角化性苔藓样丘疹性结节性损害,颜面部及头皮常表现为玫瑰痤疮或脂溢性皮炎样丘疹。皮损组织病理示苔藓样界面皮炎改变,但棘层增厚和萎缩交替出现,胶质小体大而多,且炎症浸润较扁平苔藓更深和程度更重。本病无特效治疗方法,对激素等多种药物治疗的反应差,维甲酸类药物结合光疗(NB-UVB,PUVA)或为有效治疗手段。本文就该病相关临床及组织病理学特征做一综述。     

毛囊角化病样天疱疮1例

报告1例毛囊角化病样天疱疮，患者男，63岁，胸部和腋窝起红色丘疹，水疱及棕红色湿疹样斑块1年半，组织病理检查显示毛囊角化病样表现，有棘层松解和大量角化不良细胞，间接及直接免疫荧光示表皮角质形成细胞间有IgG，及C3沉积，确诊为天疱疮。     

面颊部苔藓样角化病一例

患者，女，53岁。左侧面颊部灰褐色斑块2年余。组织病理示表皮网篮状角化过度，棘层增生肥厚，基底细胞液化变性，可见胶样小体，真表皮界限模糊，真皮内血管及附属器周边可见炎性细胞大致呈带状浸润。诊断：苔藓样角化病。患者拒绝行手术切除、液氮冷冻等治疗，给予氟尿嘧啶霜外用，半年后皮损明显好转，此后失访。     

慢性苔藓样角化病的研究进展

慢性苔藓样角化病为一种少见的角化性皮肤病,临床上表现为面部、躯干、四肢角化性丘疹、斑块,需要与扁平苔藓、扁平苔藓样角化病,红斑狼疮等相鉴别。本病治疗困难,局部使用钙泊三醇、PUVA、NB-UVB、光动力,或口服维A酸类药物,可改善病情。本文对慢性苔藓样角化病的病因、发病机制、临床表现、诊断和治疗的研究进展进行综述。     

疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。     

阿维A诱发药疹1例

正阿维A可抑制角质形成细胞增殖并调节其分化,临床上可用于治疗角化异常性皮肤病,如银屑病、扁平苔藓、扁平苔藓样角化病、汗孔角化病等。我们应用阿维A治疗1例经组织病理确诊的扁平苔藓患者,治疗20 d后出现皮疹,考虑为阿维A所诱发,由阿维A诱发的药疹国内外罕见报道,现报告如下。     

斑块型汗孔角化病1例

报道斑块型汗孔角化病1例。患者女,12岁,因双足背进行性瘙痒伴黄褐色油腻、脱屑性斑块5年余就诊,组织病理检查显示表皮内高度板层样角化过度,伴不典型角化不全柱,真皮浅层片状淋巴细胞浸润,PAS染色阴性。诊断斑块型汗孔角化病。口服阿维A治疗获得理想效果。     

表现为色素性对称性肢端角化性皮损的一组患者临床分析

目的 报道一组表现为色素性对称性肢端角化性皮损患者的临床表现、组织病理学及治疗结果。方法 系统地收集了自1998年5月至2007年9月来我科就诊的以色素性对称性肢端角化性皮损为表现的16例患者的临床资料，包括临床表现、皮损组织病理学改变、治疗、性别、年龄、职业、家族成员情况等。结果 男14例，女2例；年龄9 ~ 54岁；病程2个月至8年，平均（2.0 ± 1.5）年。主要临床表现为出现棕褐或棕黑色斑，均呈对称性斑丘疹，表面粗糙，有少许脱屑，伴轻度瘙痒。皮损部位：16例（100%）患者在双手掌指背、手腕，2例（12.5%）双肘及双膝，3例（18.8%）双踝部。组织病理学表现：表皮角化过度，棘层增厚，乳头瘤样增生；真皮少许淋巴细胞浸润。家庭成员无类似病史。给予口服维A酸类、烟酰胺、维生素B6等，外用维A酸软膏、糖皮质激素类软膏等，疗效欠佳，或病情反复。结论 这是一组主要以肢端出现对称性色素性角化斑或斑丘疹，组织病理学表现以表皮角化过度、棘层增厚、乳头瘤样增生为特征的皮肤病，病因不明。     

基底细胞癌75例临床病理分析

目的探讨皮肤基底细胞癌(BCC)组织病理特征及其鉴别诊断,以对治疗方案的选择提供指导。方法分析75例患者的临床及组织病理资料。结果按组织病理分型,实性BCC 33例,色素性BCC 14例,浅表性BCC 8例,硬斑病样BCC 5例,囊性BCC 3例,角化性BCC 4例,腺样BCC 2例,纤维上皮瘤性BCC 2例,实体与其他混合型4例。以上各型易与脂溢性角化病、光线性角化病、色痣、角化棘皮瘤、黑素细胞瘤、Bowen病等鉴别。结论各型皮肤基底细胞癌均具有明确的组织病理学特征,易与其他皮肤疾病鉴别。外科手术仍为首选治疗,Mohs显微外科手术在预防复发和美观方面更具优势,光动力疗法对表浅型BCC疗效较佳。     

水源性汗管肢端角化症一例

患者，女，26岁，因双手足遇水后反复出现丘疹、斑块10余年就诊。组织病理显示表皮角化过度，浅层血管扩张，小汗腺轻度扩张，小汗腺上皮细胞空泡样改变，符合水源性汗管肢端角化症的诊断。     

Pigmented hidrocystoma of the eccrine secretory coil in the vulva: clinicopathologic, immunohistochemical and ultrastructural studies

A case of pigmented hidrocystoma of eccrine secretory coil is presented. A 47-year-old woman had developed a bluish black small nodule in the anterior portion of the labium minor a few years before entry. Microscopically, the cyst was lined by eosinophilic columnar epithelium with abundant brownish granules. There was a vague suggestion of decapitation secretion focally in the epithelial layer of cuboidal cells. This layer expressed distinct reactivity against CA19-9 with no reactivity for human milk fat globule-1 (HMFG-1). These features demonstrated that the cyst was not of apocrine nature but of eccrine derivation. In addition, positive immunoreaction for cytokeratin (CK)7, CK8 and CK19 defined the cyst as originating from the secretory coil of the sweat gland. Ultrastructurally, melanosomes in various stages were identified in most of the epithelial cells. These findings suggest that the present case was a hidrocystoma of eccrine secretory coil with abnormal melanin accumulation.     

Paget样原位鳞状细胞癌1例

患者男,57岁。左胸偏下方皮疹4～5年。皮肤科情况:左胸部偏下方见约壹分硬币大不规则形灰褐色斑疹,表面覆有褐色痂,无破溃。皮损组织病理示:表皮角化过度,角化不全,棘层肥厚,基底层细胞完整,细胞形态大小不一,胞核大而深染,核仁明显,表皮上半部可见显著的透明细胞,类似Paget样细胞。诊断:Paget样原位鳞状细胞癌。     

The rate of corneocyte formation in microscopic lesions in patients with active psoriasis

Microscopic lesions in active psoriasis were identified after careful examination with a magnifying glass in areas free of macroscopic lesions and of hair follicles. Biopsies were excised without anesthesia, immediately frozen and cryostat-sectioned. Care was taken to preserve the horny layer which was stained to reveal corneocyte membranes and nuclei when present. The interlocking sheets of corneocyte layers made it feasible to outline and trace intercellular spaces parallel to the surface and count the number of corneocyte layers between pairs of these. It was assumed that the location of such a space in the horny layer indicates the period of time from its formation. This information was used to assess the rate of corneocyte layer formation at specified intervals of time. The result shows that adjacent areas of the horny layer could be compared and relative rates of corneocyte layer formation computed. In the parakeratotic part of the early lesions of psoriasis studied the rate of corneocyte layer formation was 15 times higher than that in the non-involved skin. The peak rate, coincident with parakeratosisj was preceded by a number of thickened corneocyte layers. These also showed an increased rate of formation, up to 6.6 times that in non-involved skin. There was no acanthosis in the lesions studied. A subepidermal cellular infiltrate was observed. Periods of rapid corneocyte formation with parakeratosis were often short (not more than one day). These were interrupted by periods with a lower rate. The relationship between corneocyte morphology and stratum corneum kinetics is a distinct feature in the early psoriatic lesion.     

Epidermal dysplasia and abnormal hair follicles in transgenic mice overexpressing homeobox gene MSX-2.

The homeobox gene Msx-2 is expressed specifically in sites of skin appendage formation. To explore its part in skin morphogenesis, we produced transgenic mice expressing Msx-2 under the control of the cytomegalovirus promoter. The skin of these transgenic mice was flaky, exhibiting desquamation and shorter hairs. Histologic analysis showed thickened epidermis with hyperproliferation, which was restricted to the basal layer. Hyperkeratosis was also evident. A wide zone of suprabasal cells were misaligned and coexpressed keratins 14 and 10. There was reduced expression of integrin beta 1 and DCC in the basal layer. Hair follicles were misaligned with a shrunken matrix region. The dermis showed increased cellularity and empty vacuoles. We suggest that Msx-2 is involved in the growth control of skin and skin appendages.     

芳香烃受体在人表皮、毛囊和皮脂腺上的表达及其意义

目的 观察芳香烃受体(AhR)在人表皮、毛囊及皮脂腺细胞上的表达及在二(噁)英作用下的激活情况.方法 采用免疫荧光和免疫组化法观察AhR蛋白在人面部表皮、毛囊、皮脂腺及体外培养的HaCaT细胞和SZ95人皮脂腺细胞上的表达情况;Western印记法观察2,3,7,8-四氯二苯-p-二(噁)英(TCDD)作用HaCaT细胞和SZ95细胞3d后AhR蛋白表达的变化情况.结果 AhR蛋白在人面部皮肤表皮、毛囊、皮脂腺细胞上表达,并且AhR蛋白在表皮棘层和颗粒层的表达强于基底细胞层,在皮脂腺小叶的外周和中央表达强于皮脂腺小叶中间,在毛囊外毛根鞘和近毛小皮细胞上的表达强于内毛根鞘细胞.AhR蛋白在HaCaT细胞和SZ95细胞的细胞核和胞质中高表达.在TCDD作用下,HaCaT细胞和SZ95细胞内AhR蛋白表达明显下降,显示AhR在TCDD作用下被激活.结论 AhR在人表皮、毛囊、皮脂腺、HaCaT细胞及SZ95细胞上高表达,并在TCDD作用下激活,显示二(噁)英有可能通过AhR信号途径引起了人表皮、毛囊和皮脂腺的异常分化.     

舌淀粉样变1例

报告１例舌淀粉样变。舌淀粉样变是由舌的粘膜下层和支内纤维状糖蛋白沉积而引起。表现为舌体肿大、有齿痕、粘膜下出血形成血肿、瘀斑、甚至溃烂、疼痛。舌背可有淡黄色蜡样颗粒。组织学切片可见舌粘膜下淀粉样物沉积，刚果红染色呈砖红色。     

392例负压抽脂微创治疗腋臭疗效分析

目的:探讨负压抽脂微创技术治疗腋臭的临床疗效及安全性.方法:收集我院皮肤科门诊腋臭患者392例,腋窝局部肿胀麻醉后使用脂肪抽吸管抽吸脂肪浅层及刮吸真皮深层,术后随访1年,观察疗效、并发症及复发情况.结果:治愈367例,治愈率93.63％,总有效率100％.其中10例出现局部硬结,2例出现上臂抬举稍受限,未见严重并发症及...     

Hypergranulotic dyscornification: a distinctive histologic pattern of maturation of epidermal epithelium present in solitary keratoses

Hypergranulotic dyscornification is an appellation proposed to designate a newly recognized distinctive pattern of epidermal maturation that is analogous to other epithelial reaction patterns such as epidermolytic hyperkeratosis, focal acantholytic dyskeratosis, cornoid lamellation, pale-cell acanthosis, and follicular mucinosis. The name "benign hypergranulotic keratosis with dyscornification" is proposed to specify solitary keratoses with digitated epidermal hyperplasia that exhibit this exceptional pattern of cornification. This abnormal type of cornification is characterized by hypergranulosis. A pale-staining basophilic substance is present intercellularly within the upper spinous layer and the hyperplastic granular layer. Overlying the thickened granular layer in foci at tips of epidermal papillations are orthokeratotic mounds of large, dull, eosinophilic staining corneocytes that are sharply demarcated from the thickened granular layer. Basophilic keratohyalin granules are focally retained within these corneocytes. There is overlying compact orthokeratosis that extends across the entire lesion. The compact orthokeratosis is slightly basophilic, and lies below a laminated and basket-weave orthokeratotic stratum corneum. There is a predominantly lymphocytic infiltrate at the base of these neoplasms with some spongiosis. There is parakeratosis focally present in the stratum corneum overlying these individual areas of abnormal cornification. The histopathologic and clinical findings in eight lesions that exhibit hypergranulotic dyscornification, a heretofore undescribed unique pattern of epidermal cornification, are presented.     

Dowling-Meara亚型单纯型大疱性表皮松解症一家系的基因突变检测

目的：研究Dowling-Meara亚型单纯型大疱性表皮松解症（DM－EBS）一家系的基因突变。方法：采用了免疫组化、电镜、聚合酶链反应－异源双链分析和DNA测序方法。结果：免疫组化显示裂隙发生于致密层以上;电镜显示本病的基底细胞下出现裂隙,张力微丝减少;通过聚合酶链反应－异源双链分析检测到K14的基因突变,进而应用直接测序法确定了突变位点：K14的1A区N123R。结论：本DM－EBS家系存在K14的基因突变。     

Proliferating cell nuclear antigen (PCNA) in common epidermal lesions.

A commercially available antibody to proliferating cell nuclear antigen was used to characterize and compare proliferating cell populations in paraffin sections of benign, premalignant, and malignant lesions of human epidermis using routine immunohistochemical techniques. Three patterns emerged. An ordered pattern was found in prurigo nodularis and keratoacanthoma, wherein moderately and strongly positive nuclei were distributed in a continuous, basal-suprabasal layer of relatively uniform thickness. There was graded loss and ultimate extinction of PCNA staining in progressively more superficial epidermal cells. A basal dysplastic pattern was found in actinic keratosis and squamous cell carcinoma. Nuclei of essentially all dysplastic cells of both categories expressed PCNA, with a preponderance of strongly positive nuclei. These were localized to basal-suprabasal zones that were often expanded. Loss of PCNA reactivity toward the surface was often abrupt. Bowen's disease exhibited a diffuse dysplastic pattern, wherein large numbers of moderately and strongly positive nuclei, in random array, were present in essentially full thickness distribution. In many fields, however, a layer of cytologically bland basal cells, with faint or no nuclear staining, was interposed between dysplastic epithelium and dermis. This study has demonstrated that proliferating cell populations in epidermal lesions can be assessed with simple, inexpensive methods. There were consistent differences between the proliferating cell populations of the various entities studied, differences that can be reasonably correlated with other known clinical, microscopic, and biologic features of the lesions. This technique should provide an interesting new avenue for study of diverse cutaneous diseases.