麻风与眼耳鼻喉畸残（附34例调查报告）

麻风与眼耳鼻喉畸残（附３４例调查报告）何瑞康（贵州瓮安县医院五官科５５０４００）李福祥（瓮安县防疫站皮防科）５５０４００麻风是由麻风杆菌引起的传染病，主要侵犯皮肤及周围神经，导致皮肤溃疡及四肢畸残。亦可继发眼耳鼻喉麻风，引起相应的病变和畴残，对病人的...     

组织细胞增多病X:局部氮芥治疗

作者报告2例皮肤组织细胞增多病X(histiocytosis X)患者,用氮芥局部治疗获得成功.例1,72岁,女,病期8年.在胸壁中部、外生殖器、头皮、腋窝、后背下部及大腿内侧发生多数瘙痒性1～2mm的红斑性和结痂性丘疹、脓疱及小疱,伴有口腔颊粘膜糜烂及溃疡.组织学检查,确定该损害为组织细胞增多病X的病变,以含有橄榄油的50cc水中溶解10mg的氮芥溶液,每日用于患处.大多数部位用塑料薄膜封闭每日5小时,擦     

皮肤利什曼病六例临床病理分析北大核心CSCD

目的探讨皮肤利什曼病的流行病学、临床及组织病理学特点。方法收集并分析6例皮肤利什曼病患者的流行病学、临床及组织病理学资料。结果6例患者均为男性,平均发病年龄47．67岁（37-67岁）,平均病程10个月（6-18个月）。皮损特点为红斑、结节、溃疡,分布于面部及四肢暴露部位。组织病理表现为感染性肉芽肿模式,组织标本中均可找到组织细胞胞质内嗜碱性小体。6例患者均具有疫区工作或旅行史。结论皮肤利什曼病的诊断主要依靠流行病资料、临床表现和组织病理学检查。     

皮肤利什曼病六例临床病理分析

目的 探讨皮肤利什曼病的流行病学、临床及组织病理学特点。方法 收集并分析6例皮肤利什曼病患者的流行病学、临床及组织病理学资料。结果 6例患者均为男性,平均发病年龄47.67岁（37 ~ 67岁）,平均病程10个月（6 ~ 18个月）。皮损特点为红斑、结节、溃疡,分布于面部及四肢暴露部位。组织病理表现为感染性肉芽肿模式,组织标本中均可找到组织细胞胞质内嗜碱性小体。6例患者均具有疫区工作或旅行史。结论 皮肤利什曼病的诊断主要依靠流行病资料、临床表现和组织病理学检查。     

系统性硬化病患者的皮肤表现及其临床意义

目的 探讨系统性硬化病（SSc）患者的皮损特点及其与临床分型、自身抗体、内脏器官损害之间的关系。方法 对2012—2014年复旦大学附属中山医院皮肤科及上海市中西医结合医院硬皮病专科确诊的120例SSc患者进行分析。结果 120例中,皮损发生率依次为雷诺现象118例（98.3%）、皮肤硬化116 例（96.7%,指背硬化101例,占84.2%）;皮肤肿胀90例（75.0%,手指肿胀84例,占70%）;皮肤异色症77例（64.2%）、口唇变薄75例（62.5%）、毛细血管扩张74例（61.7%）、口周放射性条纹63例（52.5%）、面具脸57例（47.5%）、甲小皮增生49例（40.8%）、甲皱出血点35例（29.2%）、指尖凹陷25例（20.8%）、指腹萎缩24例（20.0%）、指末节缩短24例（20.0%）、指尖溃疡15例（12.5%）。抗Scl?70抗体阳性42例（35.0%）,抗着丝点抗体阳性31例（25.8%）。手指肿胀、指尖溃疡、指腹萎缩在抗Scl?70抗体阳性组发生率高于抗Scl?70抗体阴性组（P＜0.05）。指背硬化、皮肤异色症、指尖溃疡、指腹萎缩在抗Scl?70阳性组发生率高于抗着丝点抗体阳性组（P＜0.05）。主要内脏器官损害发生率依次为肺间质病变50%（44/88）、心脏受累47.8%（55/115）、肺动脉高压35.7%（41/115）、食管受累28.3%（34/120）、肾脏受累9.2%（11/120）。弥漫皮肤型系统性硬化病（dcSSc）患者心脏受累和皮肤异色症发生率明显高于局限皮肤型系统性硬化病（lcSSc）患者（P＜0.01）。手指肿胀、指背硬化、皮肤异色症、毛细血管扩张、口唇变薄、口周放射性条纹在SSc 患者早期发生率较高,手指肿胀、指背硬化与肺动脉高压发生率相关性较高;毛细血管扩张、指尖凹陷、指尖溃疡与肺间质病变发生率相关性较高;指腹萎缩与心脏累及发生率相关性较高,差异均具有统计学意义。结论 雷诺现象、手指肿胀、指背硬化、皮肤异色症、毛细血管扩张、口唇变薄、口周放射性条纹有助于SSc早期诊断,肺动脉高压在疾病早期即会出现,指尖凹陷、指尖溃疡预示有肺间质病变,指腹萎缩预示有心脏受累。     

皮肤窦性组织细胞增生症一例并文献复习

报道1例皮肤窦性组织细胞增生症并对文献进行复习。患者,女,56岁,右上肢暗红色结节、斑块2年余,无系统受累。组织病理：真皮内大量组织细胞、淋巴细胞,可见组织细胞伸入现象。免疫组化：S-100（+）、CD68（+）、CD1a（-）。外用卤米松等治疗后皮损范围已明显缩小。     

皮肤Rosai-Dorfman病临床表现及组织病理学特征分析

目的 探讨皮肤Rosai?Dorfman病（CRDD）的临床表现、皮损形态特征和组织病理学特点。方法 收集20例CRDD患者的基本情况及临床资料,分析其皮损特征并进行分型,同时对皮损进行组织病理学检查和免疫组化染色。结果 20例CRDD患者中,多发皮损11例,单发皮损9例;按累及的解剖部位分单处16例,多处4例,共计24处。皮损表现为丘疹结节型10处（41.67%）、浸润斑块型12处（50.00％）和肿瘤样型2处（8.33％）。20例中6例皮损表现为混合型（丘疹结节型/浸润斑块型5例,浸润斑块型/肿瘤样型1例）。24处组织标本病理表现大致相同,即真皮和（或）皮下脂肪层可见数量不一、体积较大的组织细胞散在或片状分布,伴大量以淋巴细胞和浆细胞为主的炎症细胞浸润,组织细胞胞质内吞噬有数量不一的淋巴细胞、中性粒细胞等。免疫表型均为组织细胞S100阳性、CD68阳性、CD1a阴性。17处皮损病变累及真皮全层,其中13处病变侵入脂肪层;6处病变仅累及真皮浅中层;1处病变仅累及真皮深层及脂肪层。不同形态类型皮损之间的病变累及范围和炎症浸润模式均无明显差异。结论 CRDD临床皮损以丘疹结节型和浸润斑块型为主,肿瘤样型少见。不同形态皮损组织病理均可见数量不等具有伸入运动的组织细胞。S100蛋白、CD68等免疫组化染色有助于CRDD的诊断与鉴别诊断。     

维持性血液透析终末期肾病患者皮肤病变调查分析

目的　调查进行维持性血液透析（maintenance hemodialysis, MHD）的终末期肾脏病患者皮肤病变的发病情况和病变特点，探讨其危险因素。方法　对181例MHD的终末期肾病患者进行皮肤体检；收集其临床和生化资料，分析皮肤病变的患病率，病变类型及其危险因素。结果　 ①181例患者中161例（88.95％）存在不同种类的皮肤病变，其中38例（23.60％）存在1种皮肤病变，52例（32.30％）有2种，40例（24.84％）有3种、26例（16.15％）有4种、5例（3.11％）有5种。②最常见是皮肤干燥（128/181， 77.72％），其次是瘙痒（101/181，55.80％）、脱屑（73/181，40.33％）和色素沉着（70/181，38.67％）。其他有皮肤苍白（6/181，3.31％）、瘀斑（4/181，2.21％）、指（趾）干性坏疽（3/181，1.66％）、足部溃疡（2/181，1.10％）、甲纵裂（1/181，0.55％）。③皮肤病变组患者的透析龄、超滤量、血磷、钙磷乘积、甲状旁腺激素、超敏C-反应蛋白（Hs-CRP）水平，乙肝、丙肝病毒标志物阳性率明显高于无皮肤病变组；而血红蛋白、血清尿素清除指数（Kt/V）明显低于无皮肤病变组。④Logistic回归分析结果显示，透析龄、KT/V、血钙磷乘积、血甲状旁腺激素、高敏C反应蛋白（Hs-CRP）和丙肝病毒标志物阳性是MHD患者发生皮肤病变的独立危险因素。结论MHD的终末期肾病患者皮肤病变的患病率高，以皮肤干燥症和皮肤瘙痒最常见。透析龄、KT/V、血钙磷乘积、血甲状旁腺激素、Hs-CRP和丙肝病毒标志物阳性是MHD终末期肾病患者发生皮肤病变的独立危险因素。     

凤凰衣浸“920”药水治疗皮肤溃疡的临床观察

前言: 皮肤溃疡系真皮或皮肤深层之破坏所致的组织缺损,愈后有疤痕可由于各种原因引起,因为治疗不当或并发感染,往往久治不愈,易形成慢性病变,严重地影响了劳动人民的健康和工作。医用920(又叫赤霉素)是微生物赤霉菌的代谢产物,它不但能刺激植物生长,对人体尚能增强组织细胞的新陈代谢和免疫力,     

皮肤窦性组织细胞增生症1例并文献复习

报告1例皮肤窦性组织细胞增生症.患者男,64岁,背部、右下腹部结节,无痛痒9月.皮肤组织病理检查：真皮有深染区和浅染区,深染区大量浆细胞样细胞、淋巴细胞,浅染区大量组织样细胞,核淡染,核仁嗜酸性,弥漫性片状浸润,部分细胞大,有伸入现象.免疫组化染色示：S-100及AAT（＋）,CD68少量（＋）,CD1α（-）.诊断：皮肤窦性组织细胞增生症.同时对本病的临床表现、组织病理学特征及预后等情况进行分析及文献复习.     

The keratotic tumors of Cowden''s disease: an electronmicroscopic study

Cowden's disease is characterized by multiple hamartomas of the skin, breast, thyroid, and gastrointestinal tract. In the past, a viral hypothesis for the keratotic lesions of the skin has led to much controversy. The present study describes the results of a detailed fine structural analysis of 10 hyperkeratotic extremity lesions and 2 keratotic lesions from the face of a patient with Cowden's disease. Increases in the keratinocyte population were primarily confined to the basal and suprabasal regions. Differentiation products characteristic of keratinization were normal in both quantity and appearance. Nuclear remnants and numerous lipid droplets, markers of abnormal keratinization, were noted within horny cells. However, viral particles and/or virus-like particles were not observed in keratinocytes. Melanocytes and Langerhans cells were numerous. The latter contained membrane-bound pigment vacuoles in addition to the characteristic Birbeck granules. These unusual Langerhans cells were observed in the dermis as well as the epidermis. A large number of fully granulated "resting" mast cells was uniformly distributed throughout the dermis, associated with a prominent cellular infiltrate. Our observations do not support the concept of a viral etiology for these tumors.     

Activation of dendritic antigen-presenting cells expressing common heat shock protein receptor CD91 during induction of psoriasis

BACKGROUND: Psoriasis is a common and chronic relapsing inflammatory skin disorder. Although a role for T cells in mediating the induction and maintenance of psoriatic lesions is well established, mechanisms responsible for activation of T cells by antigen-presenting cells (APCs) during disease relapse are poorly understood. OBJECTIVES: (i) To determine whether expression of the common heat shock protein (HSP) receptor CD91 correlated with development of psoriasis in a mouse model of psoriasis, (ii) to characterize the lesional cells on which CD91 was expressed, and (iii) to investigate whether CD91+ cells in psoriasis showed signs of activation. METHODS: Two systems were used in order to study the above-mentioned objectives: (i) skin biopsies taken directly from patients with psoriasis (either psoriatic plaques or symptomless prepsoriatic skin) or from healthy donors, respectively, or (ii) (human) skin biopsies collected during development of psoriasis using a novel xenograft mouse model of psoriasis. The skin samples were then either processed for analysis by light microscopy, or labelled with fluorochrome-conjugated antibodies and analysed by confocal laser scanning microscopy. RESULTS: We observed a markedly increased number of CD91+ cells which paralleled development of new psoriatic lesions in the psoriasis mouse model and in established psoriatic plaques compared with symptomless prepsoriatic or healthy skin. Morphology as well as cell-specific markers showed that CD91 was predominantly expressed by dermal dendritic APCs characterized by activation of nuclear factor-kappaB signalling and the presence of tumour necrosis factor-alpha, an important proinflammatory cytokine in the immunopathogenesis of psoriasis. In addition, HSP70, a ligand for CD91, was increased in keratinocytes in close vicinity to CD91-bearing APCs in psoriatic lesions. CONCLUSIONS: These findings indicate massive presence of CD91+ dendritic cells juxtaposed to lesional keratinocytes expressing HSP70, and suggest a novel pathophysiological pathway and therapeutic target for this chronic inflammatory skin disease.     

Darier''s disease: an immunohistochemical study using monoclonal antibodies to human cytokeratins

The pathogenesis of Darier's disease was investigated by immunohistochemical staining of skin biopsies from involved and uninvolved skin in 14 patients, using monoclonal antibodies specific for keratins expressed in simple epithelia, stratified squamous epithelia and during skin specific differentiation as well as keratins expressed in mucosa and some benign epidermal hyperproliferative states. Uninvolved perilesional skin from Darier's patients showed a normal profile of keratin expression, whereas in lesional skin there was apparent delay in the expression of the suprabasal skin specific keratins. Suprabasal keratins were not detected in basal cells, thus there was no true premature keratinization. The presence of hyperproliferative keratins was restricted to suprabasal cells in lesional skin. Four patients were receiving treatment with etretinate at the time of biopsy, but results in these patients did not differ from patients using topical treatments. Etretinate did not influence the profile of keratin expression in uninvolved or involved skin. The expression of type VII collagen was examined and was normal throughout uninvolved and lesional skin in Darier's disease.     

Xeroderma pigmentosum: Diagnostic procedures,interdisciplinary patient care,and novel therapeutic approaches

Xeroderma pigmentosum (XP) is an autosomal recessive disease, caused by a gene defect in the nucleotide‐excision‐repair (NER) pathway or in translesional DNA synthesis. At the age of eight, patients already develop their first skin cancers due to this DNA repair defect. In contrast, in the Caucasian population the first tumor formation in UV exposed skin regions occurs at a mean age of 60. The clinical picture among patients suffering from XP is highly diverse and includes signs of accelerated skin aging, and UV‐induced skin cancers, as well as ophthalmologic and neurological symptoms. Patients should therefore receive interdisciplinary care. This includes dermatologists, ophthalmologists, ENT specialists, neurologists, and human geneticists. Patients with XP are clinically diagnosed, but this may be supported by molecular‐genetic and functional analyses. These analyses allow pinpointing the exact disease‐causing gene defect (complementation group assignment, detection of the type and location of the mutation within the gene). The resulting information is already relevant to predict the course of disease and symptoms and probably will be utilized for individualized therapeutic approaches in the future. Recently, enhanced repair of UV photolesions in xeroderma pigmentosum group C cells induced by translational readthrough of premature termination codons by certain antibiotics could be demonstrated.     

Functional thrombomodulin expression on epithelial skin tumours as a differentiation marker for suprabasal keratinocytes

Summary In normal human skin, immtmoreactive thrombomodulin (TM) is expressed in a strict differentiation related pattern. solely in suprabasal spinous layer keratinocytes. To evaluate the polential application of TM as a differentiation marker for keratinocyte-derived skin tumours, we have studied immunohistopathological, biochemical and functional TM activities in various skin tumours. Immunoreactive, full sized and enzymatically active TM was expressed in keratinocyte-derived skin tumours (squamous cell carcinoma, seborrhoeic keratosis and partly Bowen's disease), as well as normal epidermal keratinoeytes and endothelial cells. However, no TM was detected in basal cell carcinotnas, senile keratosis or non-squanious epithelial tumours such as malignant melanoma, naevus pigmentosus and Paget's disease. Interestingly, decreased expression was observed in verruca vulgaris. These findings suggested that differentiation-dependent TM expression was restricted to epithelial skin tumours and undetectable on neural crest derived tumours. TM is a differentiation marker for spinous layer keratinocytes and is a useful tool in histopathological study of epithelial tumours.     

Current management strategies for cutaneous T-cell lymphoma

Cutaneous T-Cell Lymphoma is a group of lymphomas characterized by a malignant proliferation of skin homing T cells. Prognosis is generally good and treatment is based on the stage of the disease with the goal of inducing remission. Patients with disease limited to the skin in the form of patches and plaques respond best to "skin directed therapy" with topical agents including corticosteroids, nitrogen mustard, carmustine, bexarotene gel, as well as phototherapy with ultraviolet B light, PUVA, or photodynamic therapy. Tazarotene and imiquimod show potential in the treatment of early CTCL. Patients with disease resistant to treatment or with advanced disease require more aggressive therapy in the form of total skin electron beam radiation, biologic response modifiers including interferon alpha, bexarotene, denileukin diftitox, extracorporeal photochemotherapy or combination therapy. The use of chemotherapy is used primarily for palliation. Allogeneic hematopoetic stem cell transplantation may represent a successful treatment for treatment resistant disease.     

Expression of monocyte chemoattractant protein-1 in the lesional skin of systemic sclerosis

Systemic sclerosis (SSc) is a connective tissue disease with unknown etiology characterized by excessive deposition of collagen in the skin as well as various internal organs. One of the characteristic histological features is the presence of infiltrating mononuclear cells in the dermis in its early stage. As well as T cells, macrophages are implicated to play an important role in the initial pathologic changes associated with SSc by releasing fibrogenic cytokines, including transforming growth factor-beta or platelet-derived growth factor. However, the precise mechanism for increased monocyte/macrophage recruitment in the lesional skin of SSc is still not completely elucidated. Monocyte chemoattractant protein-1 (MCP-1) is a predominant monocyte chemoattractant secreted by various cells types including mononuclear cells, fibroblasts, smooth muscle cells, endothelial cells, or keratinocytes. In this study, we examined the expression of MCP-1 protein and mRNA in the lesional skin of seven patients with SSc by immunohistochemistry and in situ hybridization. Results of immunohistochemistry showed that MCP-1 was detected on infiltrating mononuclear cells and fibroblastic cells in scleroderma skin, whereas normal skin showed only minimal MCP-1 expression. We demonstrated the expression of MCP-1 mRNA in infiltrating mononuclear cells and keratinocytes in scleroderma and contact dermatitis skin. In addition, signals were also detected in fibroblasts in the lesional skin of scleroderma, whereas fibroblasts in normal skin and contact dermatitis skin did not express MCP-1 mRNA. These findings suggest that MCP-1 plays a role in recruiting monocyte/macrophages in the lesional skin of scleroderma and that activated fibroblasts in scleroderma are involved in this process.     

Atopic Dermatitis

Atopic dermatitis (AD) is a chronic and relapsing disease affecting an increasing number of patients. Usually starting in early childhood, AD can be the initial step of the so-called atopic march, i.e. followed by allergic rhinitis and allergic asthma. AD is a paradigmatic genetically complex disease involving gene-gene and gene-environment interactions. Genetic linkage analysis as well as association studies have identified several candidate genes linked to either the epidermal barrier function or to the immune system. Stress, bacterial or viral infections, the exposure to aero- or food-allergens as well as hygienic factors are discussed to aggravate symptoms of AD. Athough generalized Th2-deviated immune response is closely linked to the condition of AD, the skin disease itself is a biphasic inflammation with an initial Th2 phase and while chronic lesions harbour Th0/Th1 cells. Regulatory T cells have been shown to be altered in AD as well as the innate immune system in the skin. The main treatment-goals include the elimination of inflammation and infection, preserving and restoring the barrier function and controlling exacerbating factors. The overall future strategy in AD will be aimed to control skin inflammation by a more proactive management in order to potentially prevent the emergence of sensitization as well as to design customized management based on genetic and pathophysiologic information.