盐裂皮肤直接和间接免疫荧光联合应用鉴别诊断表皮下大疱病的研究

本文探讨盐裂皮肤ＤＩＦ和盐裂皮肤ＩＩＦ联合应用鉴别诊断表皮下大疱病的价值。２０例两种盐裂皮肤免疫荧光均阳性的病例比较表明，盐裂皮肤ＩＩＦ示抗基底膜带抗体结合表皮侧或真皮侧与盐裂皮肤ＤＩＦ示免疫反应物（ＩｇＧ、ＩｇＡ、Ｃ＿３等）沉积于表皮例或真皮侧的部位基本一致。提示多数情况下盐裂皮肤ＤＩＦ或ＩＩＦ均可单独用于表皮下大疱病诊断，两者对比分析可以提高诊断率。     

盐裂皮损围围皮肤直接免疫荧光检查诊断表皮下水疱病

本文报告了应用盐裂皮损周围皮肤直接免疫荧光检查（ＤＩＦ）诊断２２例表皮下水疱病的结果，所有２２例表皮下水疱病盐裂皮损周围皮肤ＤＩＦ均显示ＩｇＧ免疫复合物沉积在表皮侧、真皮侧或表皮真皮两侧，阳性率为１００％。其中表皮侧１７例、表皮真皮两侧均有２例，此１９例均为大疱性类天疱疮（Ｂｐ）；真皮侧３例，２例为获得性大疱性在皮松解症（ＥＢＡ）、１例为大疱性系统性红斑狼疮（ＢＳＬＥ）。３例天疱疮和４例正常人皮肤均阴性。此法简便，易行，准确可靠，重复性好，值得推广使用。     

盐裂皮肤直接和间接免疫荧光联合应用鉴别诊断表上大疱病的…

本文探讨盐裂皮肤ＤＩＦ和盐裂皮肤ＩＩＦ联合应用鉴别诊断表皮下大疱病的价值。２０例两种盐裂皮肤免疫荧光均阳性的病例比较表明，盐裂皮肤ＩＩＦ示抗基底膜带体结合表皮侧或真皮侧与盐裂皮肤ＤＩＦ示免疫反应物（ＩｇＧ、ＩｇＡ、Ｃ３等）沉积于表皮侧或真皮侧的部位基本一致。提示多数情况下盐裂皮肤ＤＩＦ或ＩＩＦ均可韵用于表皮下大将民病诊断，两者对比可以提高诊断率。     

儿童获得性大疱表皮松解症(附4例报道)

儿童获得性大疱表皮松解症（ＥＢＡ）是一种自身免疫性表皮下大疱病,比较少见。本文报道４例儿童ＥＢＡ,年龄为１、４、７、１４岁,均为男性。临床上似营养不良性大疱表皮松解症２例,儿童型线形ＩｇＡ大疱病２例。３例有口腔损害,伴血疱,萎缩性瘢痕和栗丘疹。病理为表皮下水疱。ＤＩＦ均示ＩｇＧ和Ｃ３呈线状分布于基底膜带,３例还见ＩｇＡ,１例见ＩｇＭ沉积于基底膜带。盐裂ＤＩＦ示３例ＩｇＧ沉积于真皮侧。ＩＩＦ示３例Ｉ     

盐裂皮损围围皮肤直接免疫荧光检查诊断表皮下水泡病

本文报告了应用盐裂皮损周围皮肤直接免疫荧光检查（ＤＩＦ），诊断２２例表皮下水疱病的结果，所有２２例表皮下水泡病盐裂皮损周围皮肤ＤＩＦ均显示ＩｇＧ免疫复事物沉淀在表皮侧，真皮侧或表皮真皮两侧，阳性率为１００％，其中表皮侧１７例，表皮真皮两侧均有２例，此１９例的均为大泡性类天疱疮（Ｂｐ），真皮侧３例，２例为获得性大疱性表皮性松解症（ＥＢＡ），１例大疱性系统性红斑狼疮（ＢＳＬＥ），３例天疱疹和４例正常人     

疱液在诊断表皮下自身免疫性大疱病中的意义

目的：探讨表皮下自身免疫性大疱病疱液中抗基底膜带（ＢＭＺ）自身抗体的情况及在诊断中的意义。方法：应用非盐裂皮肤及盐裂皮肤间接免疫荧光技术（ＩＦ）检测３８例表皮下自身免疫性大疱病患者疱液和血清中ＩｇＧ、ＩｇＡ、ＩｇＭ、ＩｇＥ及ＩｇＧ亚型ＩｇＧ１～ＩｇＧ４抗ＢＭＺ抗体及滴度，并检测疱液和血清中结合补体Ｃ３的特异性抗ＢＭＺ抗体。结果：（１）疱液和血清中抗ＢＭＺ抗体及滴度和结合补体Ｃ３的特异性抗ＢＭＺ抗体均无显著性差异。（２）大疱性类天疱疮患者的疱液和血清中ＩｇＧ抗ＢＭＺ抗体的亚型分布相同，主要为ＩｇＧ１和ＩｇＧ４。结论：疱液可作为ＩＩＦ检测表皮下大疱病抗ＢＭＺ抗体的另一个有价值的标本来源。     

1mol／L NaCl分离表皮DIF染色法的临床实用价值

对１９例大疱性类天疱疮（ＢＰ）和５例获得性大疱性表皮松解症（ＥＢＡ）病人进行了常规ＤＩＦ、１ｍｏｌ／Ｌ ＮａＣｌ分离表皮ＤＩＦ和１ｍｏｌ／Ｌ ＮａＣｌ分离正常人皮肤ⅡＦ的对比研究。结果显示１ｍｏｌ／Ｌ ＮａＣｌ分离皮肤ＤＩＦ染色法是诊断和鉴别诊断ＢＰ和ＥＢＡ的一种简单、可靠、敏感的方法。     

红斑狼疮皮损中浸润细胞免疫表型和角质形成细胞HLA-DR抗原表达的研究

应用抗ＨＬＡＤＲ、ＣＤ３、ＣＤ４、ＣＤ８、ＣＤ２０的单克隆抗体和ｓｔｒｅｐｔｒａｖｉｄｉｎｐｅｒｏｘｉｄａｓｅｓｔａｉｎｉｎｇ（ＳＰ）技术对１０名正常人皮肤,１６例ＳＬＥ皮损和１９例ＤＬＥ皮损进行了免疫组化研究。观察到正常人皮肤角质形成细胞未见ＨＬＡＤＲ抗原表达,而ＳＬＥ（６／１６）,ＤＬＥ（８／１９）皮损处角质形成细胞可以表达ＨＬＡＤＲ抗原。在ＳＬＥ、ＤＬＥ真皮内浸润细胞主要为Ｔ淋巴细胞（ＣＤ３＋浸润细胞）,且以ＴＨ细胞（ＣＤ４＋浸润细胞）占优势。另外,还发现在两种ＬＥ表皮角质形成细胞表达ＨＬＡＤＲ抗原处,真皮内可见ＣＤ３＋浸润细胞和激活的Ｔ淋巴细胞（ＨＬＡＤＲ＋浸润细胞）。讨论了ＬＥ皮损角质形成细胞ＨＬＡＤＲ抗原表达及其与病损内浸润细胞免疫表型的关系。ＬＥ皮损处ＨＬＡＤＲ＋角质形成细胞可能具有抗原递呈作用,而角质形成细胞异常表达ＨＬＡＤＲ抗原则可能与真皮内浸润单个核细胞或淋巴细胞释放的ＩＦＮα,ＴＮＦγ等有关。     

大疱性系统性红斑狼疮的皮肤基底膜带相关抗原

免疫印迹和盐裂皮肤间接免疫荧光检测 ５例大疱性系统红斑狼疮（ＢＳＬＥ）血清，对照为 ５例获得性大疤性表皮松解症（ＥＢＡ）、２０例类天疱疮（ＢＰ）、２０例ＳＬＥ和１０例正常人血清。结果表明，３例（３／５）ＢＳＬＥ血清结合盐裂皮肤真皮侧和真皮提取物中２９０ ０００抗原，其中２例ＢＳＬＥ血清也结合表皮提取物中 １６５ ０００抗原，结果与 ＥＢＡ和部分ＢＰ血清相同。ＳＬＥ血清未结合 ２９０ ０００和 １６５ ０００抗原。提示ＢＳＬＥ血清中存在ＥＢＡ和ＢＰ抗体，推测ＥＢＡ和ＢＰ抗原可能是ＢＳＬＥ的皮肤基底膜带相关抗原。     

自身免疫性表皮下大疱病基底膜带自身抗体的检测

目的 比较免疫印迹（ＩＢ）和盐裂皮肤间接免疫荧光（ＩＩＦ）检测自身免疫性表皮下大疱病（ＳＡＢＤ）基底膜带自身抗体（ＢＭＺ－Ａｂ）的敏感性和特异怀。方法 分另应用ＩＩＦ和ＩＢ技术对９７例ＳＡＢＤ患者血清中ＩｇＧ型或ＩｇＡ型ＢＭＺ－Ｚｂ进行检测。结果 ＩＩＦ法阳性率７５．３％,免疫印迹法阳性率７９．４％,两者在检测灵敏度上无显著性差异。结论 二者均可用于ＳＡＢＤ中ＢＭＺ－Ａｂ的检测,二者联用对于提高Ｂ     

U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases

BACKGROUND: Epidermolysis bullosa acquisita (EBA) can be differentiated from other subepidermal bullous diseases by sophisticated techniques such as immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping, immunoblot and enzyme-linked immunosorbent assay. OBJECTIVES: To determine whether the diagnosis can also be made by routine direct immunofluorescence microscopy. METHODS: We studied frozen skin biopsies from 157 patients with various subepidermal immunobullous diseases. RESULTS: We found three distinct 'linear' fluorescence patterns at the basement membrane zone: true linear, n-serrated and u-serrated. The true linear pattern, often seen in conjunction with either the n- or the u-serrated pattern, was found in any subepidermal immunobullous disease with nongranular depositions. In bullous pemphigoid, mucous membrane pemphigoid, antiepiligrin cicatricial pemphigoid, p200 pemphigoid and linear IgA disease the n-serrated pattern was found, corresponding with depositions located in hemidesmosomes, lamina lucida or lamina densa. However, in EBA and bullous systemic lupus erythematosus the u-serrated staining pattern was seen, corresponding with the ultralocalization of type VII collagen in the sublamina densa zone. The diagnosis of EBA with IgG or IgA autoantibodies directed against type VII collagen was confirmed by immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping or immunoblotting. CONCLUSIONS: Using this pattern recognition by direct immunofluorescence microscopy we discovered several cases of EBA which would otherwise have been erroneously diagnosed as a form of pemphigoid or linear IgA disease.     

盐裂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 (DIF)检查 2 2例表皮下水疱病的结果 :IgG免疫复合物沉积在表皮侧 1 7例 ,表皮真皮两侧均有 2例 ,均为大疱性类天疱疮 (BP) ;真皮侧 3例 ,2例为获得性大疱性表皮松解症 (EBA) ,1例为大疱性系统性红斑狼疮 (BSLE)。对照组均阴性。此法简便易行 ,经济实用 ,准确可靠 ,值得推广使用     

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND

Immunofluorescence testing is an important tool for diagnosing blistering diseases.

OBJECTIVE

To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases.

METHODS

We retrospectively analyzed immunofluorescence results encompassing a 10-year period.

RESULTS

421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate.

CONCLUSION

Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.     

IgA-epidermolysis bullosa acquisita in a child resulting in blindness

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.     

获得性大疱性表皮松解症误诊一例并文献复习

报道一例反复误诊的获得性大疱表皮松解症并对相关文献进行复习。患者,女,25岁。皮疹泛发全身,主要表现为张力性水疱,疱壁紧张,尼氏征阴性,在外院误诊为天疱疮、线状IgA大疱病。组织病理检查示:表皮下水疱;盐裂IIF：IgG沉积在真皮侧; DIF:表皮基底膜IgG、C3、IgM、IgA带状沉积,ELISA：BP180,BP230均阴性,诊断为获得性大疱性表皮松解症,给予甲泼尼龙、氨苯砜、人免疫球蛋白、吗替麦考酚酯等治疗,病情好转。     

Psoriasis vulgaris coexistent with epidermolysis bullosa acquisita

Summary Autoimmune bullous diseases, such as bullous pemphigoid or pemphigus vulgaris. occasionally develop in psoriatic patients. In addition, a novel subepidermal bullous disease with autoantibodies against a lower lamina lucida antigen of 200kDa has recently been reported in association with psoriasis. We describe here a patient with psoriasis vulgaris who developed epidermolysis bullosa acquisita (EBA). Direct immunofluorescence revealed linear deposition of IgCl and C3 at the basement membrane zone. The patient's serum bound to the dermal side of salt-split normal human skin. However, immnumohlol analysis demonstrated that the patient's serum reacted with an EBA antigen of 290 kDa. EBA should be included in the list of autoimmune diseases associated with psoriasis vulgaris.     

Acquired subepidermal bullous diseases associated with psoriasis: a clinical, immunopathological and immunogenetic study

Summary Clinical, immunopathological and immunogenetic studies of four patients with a subepidermal bullous disease associated with psoriasis were carried out to determine the true nature of the blistering disease and to investigate further the relationship between psoriasis and acquired subepidermal bullous diseases. Autoantibodies in all four patients bound to the epidermal side of salt-split skin by indirect immunofluorescence and detected the major bullous pemphigoid (BP) antigens by immunoblotting. One had additional IgA autoantibodies binding an epidermal polypeptide of 270/280 kDa and another had circulating IgA autoantibodies which detected both BP and epidermolysis bullosa acquisita (EBA) antigens. All patients had active psoriasis at the onset of the bullous disease. Three patients were being treated with tar when blisters developed: one patient also received UVB radiation and experienced a relapse after exposure to sunlight. HLA phenotypes in three patients were determined. All the patients responded well to methotrexate. These findings demonstrate that BP is the subepidermal bullous disease most associated with psoriasis. Changes at the basement membrane zone in psoriasis may be responsible for the heterogeneous antibody response and may trigger the bullous disease, as may antipsoriatic treatment including tar and UV radiation. However, common immunogenetic mechanisms may play a crucial part in this disease association.     

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.