网状红斑性黏蛋白沉积症1例并文献复习

报告1例网状红斑性黏蛋白沉积症并复习相关文献。患者女，20岁，以面、颈、胸、背、双上臂持久性光加重性网状红斑为主要临床表现，无系统损害，相关实验室检查无异常。组织病理学示：真皮中上层较大量阿辛蓝染色阳性的黏蛋白沉积，血管周围少量淋巴细胞浸润，直接免疫荧光示基膜区IgM呈线状沉积：根据临床表现、组织病理及阿辛蓝染色等诊断为网状红斑性黏蛋白沉积症。     

变应性皮肤血管炎患者皮损内纤维蛋白的光镜与电镜观察

用普通病理、直接免疫荧光技术及电镜观察了７例变应性皮肤血管炎患者皮损内纤维蛋白，发现血管壁纤维蛋白样变性是纤维蛋白沉积的结果，纤维蛋白沉积是继发性的，但它对血管内皮细胞的损伤及血管炎的发展有重要作用。     

网状红斑性黏蛋白沉积症一例

报告1例网状红斑性黏蛋白沉积症，患者以胸背部的持久性光加重性网状红斑为主要表现，伴有点片状表面萎缩的白斑和坏死结痂，不伴系统损害，常规实验室检台无异常发现。组织病理显示．真皮有单核细胞浸润和阿辛蓝染色阳性的黏蛋白沉积.根据患者皮损的临床病理特征、组织病理及阿辛蓝染色等．诊断符合网状红斑性黏蛋白沉积症，本病临床少见。     

1例在病变部表皮下见有补体C_3沉积的寻常性天疱疮

作者报告1例用直接免疫荧光检查见表皮细胞间均有IgG沉积、表皮下层有C_3沉积的39岁女性的寻常性天疱疮。总结过去10年日本报告的天疱疮病例的结果,寻常性天疱疮患者43例中有28例(65%)有C_3沉积,有报告C_3沉积率更高。在28例中C_3沉积在表皮下层有11例(39%),而象本例一样C_3沉积在表皮下层,IgG在表皮细胞间均有的病例有9例(32%),IgG单独沉积的表皮上中层朱发生棘层松解,而IgG、C_3沉积的表皮下层则发生了棘层松解由此推测补体与寻常性天疱疮的病变发生部位有关。对于天疱疮的其他类型,落时性天疱疮有较多病例C_3沉积在表皮上层,但红斑性天疱疮大多在基底膜处有C_3沉积,与水疱发生的部位不一致。对于补体     

异位性皮炎超微病理、免疫病理改变及临床意义

目的 通过探讨异位性皮炎(AD)皮损超微病理结构和免疫球蛋白在皮损中的沉积种类，为临床治疗提供理论依据。方法 对10例AD患者皮损进行了常规病理、电镜和免疫病理的观察。结果 AD皮损常规病理表现为皮肤的亚急性和慢性炎症改变；电镜下存在免疫反应细胞相互接触现象；免疫球蛋白亚类的沉积以IgG1为主，沉积部位主要为真皮乳头区域。结论 细胞间的相互接触现象可能为皮肤免疫应答反应的细胞形态学基础，而皮损中IgG1的沉积可能与皮肤的感染有关。     

成人与儿童线状IgA大疱病的比较观察

本文对免疫病理表现有线状IgA沉积在BMZ的24例患者(成人18例、儿童6例)进行了临床、病理与免疫病理的分析观察,发现成人与儿童在临床症状、皮损形态与分布以及免疫病理等方面均有不同之处,说明成人与儿童线状IgA大疱病是线状IgA大疱病病谱中的两个分立疾患.本文也对免疫球蛋白在BMZ的沉积型进行了分析,发现除了线状沉积外,其中有2例成人的沉积型为颗粒状线状,而且既沉积在BMZ,也沉积在真皮乳头.认为这两例应考虑为(DH)与(BP)的混合型.另1例呈颗粒状线状沉积在BMZ,考虑为IgA BP.     

盐裂间接免疫荧光技术在大疱性类天疱疮鉴别诊断中的应用研究

目的:探讨盐裂皮肤间接免疫荧光(IIF)技术在大疱性类天疱疮(BP)鉴别诊断中的作用.方法:应用盐裂IIF技术检测78例常规方法诊断为BP的患者血清.结果:43例血清IgG沉积于表皮侧,7例IgG沉积于双侧,11例IgG沉积于真皮侧,另有17例双侧均未见抗体沉积.结论:盐裂IIF仅能用于BP的初步鉴别诊断.     

麻风皮疹的直接免疫荧光观察

用直接免疫荧光法,观察了27例各型麻风病人皮疹的免疫病理特征。结果:2例 BT,基底膜区(BMZ)有 IgG、IgM 沉积呈带状。1例 BZ,BML 部位有 IgM 沉积。从 BT—BL 的大多数病例,其皮内血管壁上有 IgM、C_3沉积,IgA 全部病例阳性;2例Ⅰ,3例 TT,未见有 Ig 沉积。这对麻风发病机理的研究将有重要意义。     

盐裂解皮损周围皮肤直接免疫荧光鉴别诊断表皮下水疱病

应用盐裂皮损周围皮肤直接免疫荧光检查２２例表皮下水疱病的结果：ＩｇＧ免疫复合物沉积在表皮侧１７例，表皮真皮两侧均有２例，均为在性类天疱疮，真皮仙３例，２例为获得性大疱性表皮松解症，１例为大疱性系统红斑狼疮，对照组均阴性。此法简易易行，经济实用，准确可靠，值得推广使用。     

自身免疫性大疱性皮肤病患者血清中IgM抗体所识别的皮肤抗原的研究

目的:研究自身免疫性大疱性皮肤病患者血清中IgM抗体所识别的皮肤抗原成分.方法:该研究共纳入10例大疱性类天疱疮患者、15例天疱疮患者(包括7例红斑/落叶型和8例寻常型).首先通过直接免疫荧光法分析受试患者皮肤中所沉积的免疫球蛋白或补体,再通过免疫印迹方法分析患者血清中的IgM抗体所识别的皮肤抗原成分.结果:在10例大疱性类天疱疮患者的皮肤中,C3、IgG、IgM单独沉积的例数分别是4例、2例、1例,IgG和C3共同沉积的2例,IgG、C3和IgA三者共同沉积的1例;在15例天疱疮患者中,C3、IgG单独沉积的例数分别是4例和2例,IgG和C3共同沉积的6例,IgM和C3共同沉积的3例.免疫印迹研究发现9例(9/10)大疱性类天疱疮患者、11例(11/15)天疱疮患者血清中的IgM抗体可以识别皮肤中分子量约80 kD的蛋白质.结论:IgM在自身免疫性大疱性皮肤病患者的皮肤中沉积的几率很低,但大多数患者血清中的IgM抗体都能够识别分子量约80 kD的皮肤抗原.     

Wellness,burnout, and the dermatologist: Fundamentals for practice longevity

Physician wellness is a critical component of any effective health care system, as physicians serve essential roles as diagnosticians, surgeons, and leaders in medical care. Physician burnout, defined as a combination of the presence of emotional exhaustion, depersonalization, and a diminished sense of personal accomplishment, is an increasingly recognized problem in the US health care system, as rates of burnout among physicians are on the rise, now exceeding 50%. To date, few studies have examined the impact of burnout on dermatologists specifically, but existing studies evaluating physicians collectively have shed light on the problem that exists in our specialty. This review focuses on the causes of physician dissatisfaction and burnout and provides an overview on interventions to mitigate them while emphasizing wellness; where applicable, special emphasis is placed on dermatologists.     

Mechanism of mediators in human skin. Pharmacoanalysis and therapeutic aspects]

Under physiological and pathological conditions, the skin is influenced by tissue hormones (biogenic amines, kinines, prostaglandines), neuro-hormones (acetylcholine, catecholamines) and by proteins as complement. The combination of neurological stimulation with a series of self-propagating metabolic changes, shows new aspects of tissue reaction. Histamine triggers a serie of mediators as kinines and prostaglandines. Due to their influence on mediators and their effects on regulatory mechanisms the cyclic nucleotides can serve as model substances for the development of therapeutic processes for dermatological as well as for other disorders. The influence on mediator-systems by topical treatment and proven permeability of such substances is of extreme importance for the future development of local dermatological therapy.     

A trip through the signaling pathways of melanoma

Many cellular signaling pathways are involved in the development of cancer. Depending on the tumor entity, the nature as well as the mode of activation can differ. Some signaling pathways frequently show changes as all tumor cells have to fulfill some basic requirements such as independence from growth factors or insensitivity against apoptosis. In this review, the possibilities of a tumor to manipulate signaling pathways to reach these goals are exemplified based on an archetypical melanoma cell. In addition, new therapeutic options based on the knowledge of signaling pathways will be discussed.     

蕈样肉芽肿的发病机制及治疗进展

蕈样肉芽肿的发病机制目前仍不清楚,近年来对蕈样肉芽肿皮肤淋巴细胞归巢机制、淋巴细胞凋亡异常以及基因异常的研究,为揭示蕈样肉芽肿发病以及治疗提供了一些新的观点。除传统的光化学疗法、放疗、化疗、体外光化学疗法外,维A酸类、干扰素、白介素2融合蛋白、重组白介素12、人源化单克隆抗体等免疫调节剂对早期蕈样肉芽肿有较好的疗效。     

Effects of extracellular matrices on human keratinocyte adhesion and growth and on its secretion and deposition of fibronectin in culture

Extracellular matrices (ECMs) play an important role as components of basement membrane of normal human skin and in migrating epidermal cells in wound healing. We investigated the effects of various ECMs on human keratinocyte adhesion and growth as well as on its secretion and deposition of fibronectin (FN) in vitro using a serum-free, low-calcium culture system. Since cell adhesion is the first step of cell growth, we performed cell adhesion assay for 14 h. Human keratinocytes adhered best on FN and less well on types I/III collagen, type IV collagen, and heparan sulfate proteoglycan (HSPG) as compared with bovine serum albumin (BSA) (control) or laminin (La). Cell growth assayed for 7-8 days on the dishes coated with various extracellular matrices revealed significantly increased keratinocyte growth on FN and on types I/III collagen in comparison with that on type IV collagen, HSPG, BSA (control), or La. Morphology of keratinocytes and of their colonies on FN and types I/III collagen was strikingly different from that of the control; the colonies were not so compact as in the control, but rather loose and larger; each keratinocyte was spread out more on these substrata. These morphologic features seemed to correlate with the increased keratinocyte growth on these extracellular matrices. Both immunofluorescence study for FN with keratinocytes in 8-day culture on various extracellular matrices and enzyme-linked immunosorbent assay for FN measurement on substratum or in conditioned medium with keratinocytes in 5-day culture demonstrated that extracellular matrices modulated the secretion and deposition of FN by human keratinocytes in culture; the keratinocyte growth correlated with the amount of FN detected on substratum but not with that in medium. Based on the results of the present investigation, we think that the growth of human keratinocytes depends on the amount of FN on substratum.     

The clinical spectrum of psoriasis

The clinical picture of psoriasis is not uniform. Being one of the most common chronic inflammatory skin disorders, psoriasis may present in many different forms and may include extracutaneous manifestations. Classifications have been proposed based on disease onset or the clinical course of psoriasis. Chronic plaque psoriasis occurs in a variety of clinical forms primarily distinguished by size, distribution, and dynamics of psoriatic plaques. In addition, psoriasis inversa, localized and generalized pustular forms, erythrodermic psoriasis, as well as a number of more uncommon forms have been recognized, a distinction on clinical grounds that is relevant for the overall prognosis and impact on the patients' quality of life as well as for the choice of therapy.

The broad and rather colorful clinical spectrum of psoriasis as well as implications for clinical practice will be comprehensively reviewed in this article.     

Pemphigus erythematosus

Pemphigus erythematosus, also known as Senear-Usher syndrome, was originally described as a variant of pemphigus with features of lupus erythematosus but regarded today as a localized form of pemphigus foliaceus and considered an autoimmune bullous disease. The autoantigen is desmoglein 1, a desmosomal adhesion protein in keratinocytes. A 69-year-old man presented with a 3-month history of erosions and blisters on the cheeks, which then also appeared on the trunk. Clinical and histopathologic criteria as well as immunofluorescence studies lead to the diagnosis of pemphigus erythematosus with transition to pemphigus foliaceus.     

Lack of host cellular immune response in eruptive molluscum contagiosum

A lack of cellular immunity on the part of the host has been incriminated as the cause of the persistence of the cutaneous lesions of molluscum contagiosum. We present a patient in the eruptive phase of the disease, confirming the absence of T-lymphocyte and natural killer cell subsets in the base of these typical lesions, using a panel of monoclonal antibodies. We also report the observation of lipid material ultrastructurally (confirmed by osmium staining on fresh-frozen tissue), as well as cross-reactivity immunocytochemically of the antigens on these molluscum bodies with antigens normally present on macrophages, as defined by DAKO-macrophage monoclonal antibodies. We have considered the possible role of these findings in the lack of host cellular responsiveness in the eruptive phase of the disease.     

Hyaluronan-independent adhesion of CD44H+ and CD44v10+ lymphocytes to dermal microvascular endothelial cells and keratinocytes.

We have recently shown the CD44 variant isoform 10 (CD44v10) to be expressed on reactive as well as malignant cutaneous lymphocytes; however, the functional consequences of CD44v10 expression on lymphocytes are not elucidated. By using appropriately transfected lymphatic cells we analyzed the role of CD44v10 on lymphocytes in cell-matrix adhesion and homotypic and heterotypic cell-cell adhesion assays. Despite a low binding affinity to hyaluronan, CD44v10-expressing lymphocytes exhibited heterotypic cell-cell adhesion to inflamed dermal microvascular endothelium and keratinocytes, as indicated by Stamper-Woodruff assays on tissue sections of delayed type hypersensitivity reactions and adhesion assays with cultured keratinocytes and cytokine-stimulated human dermal microvascular endothelial cells. Antibody-blocking assays excluded interaction of CD44v10 with the principal CD44 ligand hyaluronan as well as involvement of selectins or integrins in these heterotypic cell-cell adhesion assays. In contrast, cellular aggregation assays with fluorescence-labeled CD44v10- and CD44H-expressing lymphocytes revealed homotypic CD44v10/CD44v10 binding as well as binding of CD44v10 with CD44H. Heterotypic cell-cell adhesion assays with ultraviolet-A-irradiated CD44v-negative cytokine-stimulated endothelial cells demonstrated binding kinetics of CD44v10-expressing lymphocytes paralleling those of endothelial CD44H expression. These results imply that a hyaluronan-independent CD44v10/CD44H-mediated pathway is involved in lymphocyte infiltration into the dermis and epidermis of inflamed skin and suggest modulation of CD44H expression on inflamed dermal microvascular endothelium as a mechanism of ultraviolet-A-induced therapeutic effects on the skin.     

Retinol binding protein in normal human skin and in cutaneous disorders

In order to investigate the molecular basis of substrates, and the inhibitory specificity of tyrosinase, a large series of phenolic compounds have been analysed by using a High Performance Liquid Chromatographic-Scanning Spectrophotometric system. Depending on their chemical structure, phenolic compounds may act as substrates or as competitive inhibitors of tyrosinase. The ability to act as substrates requires the presence in the molecule of electron donor groups, while competitive inhibition on the contrary requires the presence of powerful electron acceptor groups. Certain phenolic compounds used as therapeutic agents or as food preservatives are chemically capable of acting as alternative substrates or competitive inhibitors of tyrosinase in vitro; their effect on melanocytes in vivo therefore merits investigation.