一例Sweet综合征的免疫病理研究

本文报告一例经免疫病理研究发现于皮损处真皮上层有带 IgM 和 IgE 的嗜中性白细胞,周围血象中嗜中性白细胞的趋化性有缺陷。患者女性68岁,上感后一周发生结膜炎、发热和皮损,皮损为红色隆起斑块、疼痛,分布于右颧、前臂和右小腿。化验:ESR80mm/小时(韦氏法),WBC20000,嗜中性85%,淋巴14%,单核1%。蛋白电泳和 IgG、IgM、IgA、IgE 值均正常。抗链“O”:1:800。无循环免疫复合物检出。皮损组织象示:真皮全层直至皮下密集嗜中性白细胞浸润,部分变性和核破碎和核固缩,仅见少量淋巴细胞和浆细胞。真皮中部可见扩张的小血管。给于强     

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。     

发疹型药疹皮损内免疫球蛋白和补体C3的免疫组化检测

为研究体液免疫与发疹型药疹发病机制的关系,用免疫组化PAP法对34例发疹型药疹患者皮损组织中免疫球蛋白IgG、IgM 、 IgA、IgE和补体C3进行了检测。结果真皮浅层及中层的小血管壁及管腔中和胶原纤维有IgG、IgM、IgA及C3的沉积,无IgE的沉积。结果示体液免疫在发疹型药疹的发病中起重要作用。     

皮肤异色病样淀粉样变性免疫荧光及电镜观察

报告1例皮肤异色病样淀粉样变性.患者男,40岁.四肢、躯干色素沉着和色素减退相间,伴苔藓样丘疹及毛细血管扩张20年,无自觉症状.全身皮损于夏季加重且可出现水疱.家族中无类似疾病史,父母系近亲结婚(表兄妹).皮损组织病理检查示真皮乳头内有红色团块样均质性物质,结晶紫染色阳性.直接免疫荧光(DIF)检查:表皮基膜带及表皮细胞间IgG、IgM、IgA、C3均阴性,真皮乳头及真皮浅层可见大量团块状物质沉积,其中IgG标记可见较强荧光.电镜检查:真皮乳头及真皮浅层可见大量团块状物质沉积与基膜带及基底细胞关系密切.     

大疱性系统性红斑狼疮五例分析

目的 探讨大疱性系统性红斑狼疮的临床及病理特征,提高对该病的认识.方法 对5例大疱性系统性红斑狼疮患者皮损进行组织病理检查,并用鼠抗人免疫球蛋白(IgG,IgM,IgA)及补体C3进行直接免疫荧光检查,对临床、组织病理特点及治疗预后情况进行分析.结果 皮损好发于面部、上肢等曝光部位,临床表现为正常皮肤上出现水疱或者大疱,5例患者病理的典型改变为表皮下水疱,水疱内及真皮乳头可见较多中性粒细胞浸润.5例患者免疫病理均显示免疫球蛋白和(或)补体在基底膜带呈带状沉积.患者对氨苯砜治疗反应较好,糖皮质激素联合免疫抑制有效.结论 大疱性系统性红斑狼疮可能与狼疮肾炎活动有关.氨苯砜联合糖皮质激素或者糖皮质激素联合免疫抑制剂治疗有效.     

寻常性银屑病并发成人型线状IgA大疱性皮病

报告l例寻常性银屑病并发成人型线状IgA大疱性皮病.患者男,36岁.因全身红色斑疹伴白色鳞屑反复发生20年.躯干、双上肢出现环状排列的水疱10d伴瘙痒就诊.皮损组织病理检查:表皮下水疱,疱内、真皮浅层和真皮乳头见中性粒细胞、嗜酸性粒细胞浸润;皮损周围皮肤直接免疫荧光显示基膜带Iga、IgG呈带状沉积;取患者血清行BP180NC16A(大疱性类天疱疮18 000抗原的近膜片段)-ELISA检查显示阴性;以盐裂正常人皮肤为底物,取患者血清行间接免疫荧光检查显示IgA、IgG呈带状沉积在真皮侧.诊断为寻常性银屑病并发成人型线状TgA大疱性皮病.     

疱疹样皮炎10例临床、组织病理及直接免疫荧光特征分析

目的 探讨疱疹样皮炎的临床表现、组织病理及直接免疫荧光特征。方法 对2015年1月-2020年9月西京皮肤医院确诊的10例疱疹样皮炎的临床及病理资料进行回顾性分析。结果 男6例,女4例,发病年龄为12～74岁,平均发病年龄(53.60±20.97)岁。10例患者皮损以四肢(肘部、膝部)及臀部为主,对称分布,其中有4例累及头面部。1例患者面部水疱,背部及上肢为荨麻疹样红斑。所有患者均伴剧烈瘙痒。组织病理：8例可见真皮乳头中性粒细胞微脓肿,7例见表皮下疱。直接免疫荧光显示10例患者真皮乳头IgA颗粒状沉积,其中9例患者同时伴有真表皮交界处IgA颗粒状沉积。3例真皮浅层纤维状IgA沉积。结论 疱疹样皮炎皮损好发于肘部、膝部及臀部,对称分布,剧烈瘙痒,病理表现为真皮乳头中性粒细胞微脓肿。直接免疫荧光显示真皮乳头及(或)真表皮交界处有IgA颗粒状沉积,可伴真皮浅层纤维状IgA沉积。     

大疱性系统性红斑狼疮1例

分析报告1例大疱性系统性红斑狼疮。患者以全身皮肤粘膜起红斑、水疱为表现起病，伴有口腔溃疡、双膝关节疼痛，尿蛋白3 ，ANA阳性1：160颗粒型，ds-DNA抗体阳性，ENA阳性、SS-A阳性。取躯干部皮损行组织病理活检，皿染色示：表皮下水疱，真皮浅层水肿，真皮乳头见中性粒细胞聚集，免疫病理示基底膜带可见IgG呈颗粒状沉积，符合大疱性系统性红斑狼疮病理改变。该病例为大疱性系统性红斑狼疮。     

结节性类天疱疮七例临床病理分析

目的 回顾分析结节性类天疱疮的临床特点。方法 回顾分析7例结节性类天疱疮患者的性别、发病年龄、临床表现及治疗和随访情况。结果 7例结节性类天疱疮患者中,女4例、男3例,发病年龄中位值59岁。临床表现以痒疹样皮损、结节为主,伴或不伴水疱,瘙痒症状明显,确诊前均误诊为结节性痒疹、湿疹。所有患者病理表现均有表皮增生肥厚,可见表皮下裂隙,真皮乳头胶原增生,浅层血管周围淋巴细胞、嗜酸性粒细胞浸润;直接免疫荧光检查基底膜带IgG和C3呈线状阳性,间接免疫荧光有2例阳性。7例联合使用糖皮质激素和免疫抑制剂治疗有效。结论 结节性类天疱疮临床易误诊,免疫病理检查有利于诊断,糖皮质激素和免疫抑制剂治疗有效。     

皮肤淀粉样变皮疹的直接免疫荧光观察

本文报告应用直接免疫荧光技术,观察21例皮肤淀粉样变的免疫病理特征.发现在邻近表皮的真皮,特别是乳头部位,有免疫球蛋白和补体的沉积,少数病例IgG或IgM沿着表皮、真皮之间或基底膜带部位沉积.并讨论了可能的病理诊断上的意义.     

重组腺病毒载体转染 CTLA4Ig基因在毛乳头细胞中的表达

目的：探讨免疫耐受基因（CTLA4Ig）在毛乳头细胞中的表达,为毛乳头细胞移植后产生免疫耐受提供理论和实验依据。方法：通过构建的重组腺病毒载体将免疫耐受基因（CTLA4Ig）cDNA导入培养的毛乳头细胞中,并将体外基因转染的毛乳头细胞移植到小鼠体内,通过组织学和免疫组化检测目的基因在体内和体外的表达。结果CTLA4Ig蛋白在基因转染6h后即在细胞浆中表达,随着转染时间的延长其表达逐渐增加;基因转染的毛乳头细胞移植到小鼠体内,目的基因在24h开始表达,能持续表达2周;移植细胞未受到免疫排斥。结论：带有CTLA4Ig基因的毛乳头细胞在体外和小鼠体内能较长时间存活并表达CTLA4Ig。     

Celiac Disease in Children with Atopic Dermatitis

Celiac disease (CD) is an autoimmune disorder of the small intestine with highly variable clinical presentation and frequently associated with various immune‐mediated diseases. Among these immune‐mediated diseases, atopy has been found frequently in individuals with CD. We aimed to study the prevalence of CD in Estonian children with atopic dermatitis (AD), a common multifactorial chronic inflammatory skin disease. We recruited 351 consecutive children with active AD (mean age 5.8 yrs, 57.6% boys) at Tallinn Children's Hospital, Estonia. Sera of all patients were tested for total serum immunoglobulin (Ig) A, for IgA‐ and IgG‐type autoantibodies to tissue transglutaminase (IgA‐anti‐TG2, IgG‐anti‐TG2) and to deamidated gliadin peptides (IgA‐anti‐DGP, IgG‐anti‐DGP). The diagnosis of CD was confirmed histologically by small intestine biopsy according to the European Society of Paediatric Gastroenterology, Hepatology and Nutrition diagnostic criteria. IgA deficiency was detected in nine patients with AD (2.6%), none of whom had IgG‐anti‐TG2 or IgG‐anti‐DGP seropositivity. IgA‐anti‐TG2 positivity was found in 4 (1.1%), IgG‐anti‐TG2 positivity in 2 (0.6%), IgA‐anti‐DGP positivity in 11 (3.1%), and IgG‐anti‐DGP in 10 (2.8%) patients. Celiac disease was confirmed in five (1.4%) patients with AD (95% confidence interval 0.46, 3.32) and all were histologically characterized as Marsh IIIa–IIIc stages and two presented with silent CD. In AD patients, CD prevalence was more than four times as high as in previously studied randomly selected schoolchildren in Estonia. Two patients with AD diagnosed with CD had no symptoms indicative of CD, in spite of extensive histologic changes in the small intestine mucosa. Therefore our study emphasizes the need for evaluating the cost‐effectiveness of screening individuals with AD for CD in time to prevent long‐term complications.     

Skin immunoglobulin deposition following intravenous immunoglobulin therapy in toxic epidermal necrolysis

Human intravenous immunoglobulins (IVIg) which contain anti-CD95 antibodies have been proposed to treat toxic epidermal necrolysis (TEN). Presently, there is no evidence that IVIg reach the keratinocytes in TEN patients. The aim of this study was to assess the Ig distribution in the serum, blister fluid and skin of six consecutive TEN patients treated with IVIg (1 g/kg/day) for 3 days. They were compared with five TEN patients who only received supportive therapy. In all patients, IgA, IgM and IgG concentrations were measured in the serum and blister fluid using an immuno-nephelometric method. Immunohistochemistry was performed on skin biopsies taken from both TEN clinically involved and uninvolved skin to search for IgG deposits. On admission, the IgG concentrations were significantly higher in both TEN serum and TEN blister fluid compared with their respective IgA and IgM contents. The IgG, IgA and IgM concentrations in blister fluid were significantly lower than their respective serum concentrations. The serum and blister fluid IgG concentrations, but not that of IgA and IgM, were markedly increased at the completion of the IVIg treatment. By contrast, they remained unchanged in the TEN patients that were untreated with IVIg. In the IVIg-treated patients, the IgG intraepidermal deposits raised markedly in both TEN-involved and uninvolved skin. This was not the case in patients who did not receive IVIg. These results suggest that IVIg perfusions brought a prominent increase in IgG concentration in the serum, blister fluid and epidermis of both TEN-involved and clinically uninvolved skin. The presence of potentially protective IgG in TEN epidermis following IVIg treatment could help limiting the disease progression.     

Clinical analyses of atopic dermatitis in the aged

The aim of the present study was to analyze the characteristics of atopic dermatitis (AD) in the senile phase. Subjects were comprised of 16 patients investigated for clinical features, serum immunoglobulin (Ig)E levels and skin manifestations. Mean age was 76.9 +/- 6.2 years (range, 68-87), with a man : woman ratio of 3:1. Mean age at onset was 67.7 +/- 15.7 years. Eight patients (50%) had personal histories of chronic eczema until the young adult phase and three patients (18.8%) showed the classic course of child AD. Eczematous erythroderma in 10 patients (62.5%) and unclassified chronic eczema in five patients (31.3%) were the predominant clinical presentations. Mean total IgE level in sera of the 16 patients was 8810 +/- 13 511 IU/mL (range, 5-53 605). Fourteen patients showed positive results for antigen-specific IgE antibodies, and the mean total IgE level for these patients was 10 056 +/- 14 044 IU/mL. Specific IgE to the main antigen, Dermatophagoides farinae, was observed in 12 patients (85.7%), representing the principal antibody in eight patients (57.1%). Eczematous dermatitis manifested predominantly in the face and neck, trunk and extensor and flexure sites of extremities, and less commonly in the antecubital and popliteal areas. Other stigmata of AD were observed as follows: red face in 10 patients (62.5%); Hertoghe's sign in six (37.5%); goose-skin in four (25%); facial pallor in three (18.8%); and dirty neck in one (6.3%). These results indicate that senile-type AD represents a characteristic subgroup of AD that appears in the last stage of life in AD patients.     

Serum antibodies to wheat germ agglutinin and gluten in patients with dermatitis herpetiformis

Summary It has been speculated that gluten may play a role in the pathogenesis of dermatitis herpetiformis (DH) because it can act as a lectin. The lectin activity of gluten preparations was recently identified as wheat germ agglutinin (WGA). IgG and IgA serum antibodies to WGA and gluten were therefore measured in patients with DH and coeliac disease (CD) by an enzylac-linked immunosorbent assay (ELISA). Compared with healthy controls, both patients categories had increased IgG and IgA activities to WGA and gluten, the CD group showing the highest antibody levels. DH patients with subtotal villous atrophy tended to have higher activities than those with no villous changes or only minor changes. No significant difference in the gluten-to-WGA ratio of IgA or IgG antibodies was found when DH patients were compared with CD patients. If WGA plays a pathogenetic role in DH, then DH patients must have dermal characteristics, as yet undefined, that explain the initiation of their skin disease.     

A young man with hyperimmunoglobulin-E syndrome and IgA and IgG deficiencies

Hyperimmunoglobulin E syndrome (HIES) with recurrent infection is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses, cyst-forming pneumonia and an elevated serum immunoglobulin (Ig)E level. We report an 18-year-old man with recurrent chest infections, skin infections and dermatitis. On examination, he had the characteristic facies of HIES: high arched palate, webbing between his thumb and index finger bilaterally, and extensive scarring from multiple staphylococcal skin abscesses. He had an elevated IgE level of 14 300 kU/L. IgA and IgG deficiencies were also identified, which are rare associations of this syndrome and complicated the patient's treatment. The coexistence of HIES, IgA and IgG deficiencies has, to our knowledge, not been reported previously in the literature.     

Linear IgA disease: the IgA and IgG response to dermal antigens demonstrates a chiefly IgA response to LAD285 and a dermal 180-kDa protein

BACKGROUND: Linear IgA disease (LAD) of adults and children is mediated by IgA antibodies that target proteins of the epithelial adhesion complex. Most studies have concentrated on the epidermal-associated antigens; the dermal antigens remain unresolved. OBJECTIVES: To determine the dermal antigen repertoire of IgA and IgG antibodies in LAD. METHODS: Immunoblotting was carried out on salt-split and urea-extracted dermal skin extracts with IgA antibodies (63 adult and 34 childhood sera) and with IgG antibodies (49 adult and 18 childhood sera). RESULTS: Antigens were identified by IgA (61%), IgG (27%) and by both antibody isotypes (19%). LAD285 and an antigen of 180 kDa were the major dermal antigens identified, and antigens of 230 kDa, collagen VII and a protein under 100 kDa were identified less commonly. IgA autoantibodies from adults bound single antigens more frequently than multiple antigens; from children they bound single and multiple antigens equally. The binding of multiple antigens was, however, more common in children than adults. The IgG response was weaker. The 180-kDa antigen was the main IgG dermal target, and with a single exception, IgG autoantibodies targeted single antigens. CONCLUSIONS: There was an IgA and IgG response to dermal antigens in LAD; however, the dual antibody response was limited. The antibody response to LAD285 and a 180-kDa antigen (probably BP180) suggests that intermolecular epitope spreading of the antigens associated with the extracellular matrix/dermal components of the basement membrane contributes to the immunopathology of the disease. The restricted IgG response suggests that dermal-binding IgG autoantibodies are not pathologically significant.     

SERUM IMMUNOGLOBULINS IN PSORIASIS BEFORE AND AFTER ULTRA VIOLET LIGHT THERAPY

SUMMARY.— A significant increase of the mean IgA globulin value was noted in 105 patients with ordinary psoriasis whereas the mean IgG and IgM globulin levels remained normal. Thirty-two of these patients received u.v. light therapy which resulted in resolution of the skin lesions. At this stage the IgA levels fell to the normal range. The possibility of the skin eruption influencing the IgA serum levels is discussed.     

Immunoglobulins and Their Receptors on Epidermal Langerhans Cells in Atopic Dermatitis

To elucidate the etiological role of immunoglobulin molecules on Langerhans cells (LCs) in atopic dermatitis, we conducted immunohistochemical studies on the localization of immunoglobulin G1 (IgG1), IgG2, IgG3, IgG4, IgA and IgM on epidermal LCs from 30 patients with atopic dermatitis (AD) and five non-atopic healthy volunteers. We also investigated the types of receptors for the immunoglobulins (FcεRI, FcεRII, FcγRI, FcγRII, and FcγRIII) on epidermal LCs in the patients. IgE positive epidermal LCs were observed in 28 of 30 AD patients, and 46.7% of the epidermal LCs were positive for IgE. Both IgG1- and IgG2-positive epidermal LCs were obserbed in 70% of AD patients, and 21.8% and 28.7% of the total epidermal LCs were positive for IgG1 and IgG2, respectively. IgG3- or IgG4-positive LCs were present in only small proportions of AD patients. IgA-positive LCs were observed in 8 AD patients; our study suggested that the IgA bound on LCs was secretory IgA (S-IgA). These surface immunoglobulins were observed significantly more frequently on epidermal LCs in the involved skin of AD than in clinically uninvolved skin. No IgM-positive epidermal LCs were observed in the AD patients or healthy volunteers. In non-atopic healthy controls, no immunoglobulin-binding LCs were observed. In receptors for immunoglobulins, FcεRI and FcγRII were exclusively expressed on nearly all epidermal LCs from all AD patients and all non-atopic controls. These results suggested that not only IgE but also IgG and IgA may play some etiological role in the pathogenesis of AD.     

Circulating IgA and IgE autoantibodies in antilaminin‐332 mucous membrane pemphigoid

Background Antilaminin‐332 mucous membrane pemphigoid (MMP) is a chronic autoimmune bullous disease that is often associated with internal malignancy. IgG autoantibodies against laminin‐332 in patients with MMP are well documented; however, IgA and IgE autoantibodies against laminin‐332 have not yet been described. Objectives To characterize IgA and IgE autoantibodies binding to laminin‐332 in sera from patients with antilaminin‐332 MMP. Methods Sera and skin samples from four patients who met the following criteria were used: (i) subepidermal blistering lesions present on the mucous membranes; (ii) in vivo deposition of IgG along the epidermal basement membrane zone of sampled skin; (iii) circulating IgG antibasement membrane zone antibodies that react with the dermal side of salt‐split normal human skin; and (iv) circulating IgG autoantibodies that do not show positivity against type VII collagen or 200‐kDa protein (p200 antigen) in immunoblot analysis using dermal extracts. Circulating IgG/IgA/IgE class autoantibodies against laminin‐332 were determined by immunoblotting. Results Circulating IgG autoantibodies against the γ2, α3/γ2, α3 and α3/β3/γ2 subunits of laminin‐332 were demonstrated in sera from four patients, respectively. Serum from one of the four patients showed IgA reactivity with the α3/β3/γ2 subunits of laminin‐332. Serum from one of the four patients showed IgE reactivity with the γ2 subunit of laminin‐332. The control sera failed to display IgG/IgA/IgE reactivity to laminin‐332. Conclusions In addition to IgG autoantibodies, circulating IgA and IgE autoantibodies against laminin‐332 are detectable in a subset of patients with antilaminin‐332 MMP.