大疱性类天疱疮患者发根毛囊中抗体的检测

目的：通过检测大疱性类天疱疮患者拔出的头发上残留毛囊中大疱性类天疱疮抗体的沉积情况,建立一种快捷、损伤小的检测大疱性类天疱疮的方法。方法：直接免疫荧光方法测定35例大疱性类天疱疮患者拔出的头发上残留毛囊中类天疱疮抗体的沉积,10例健康人及20例天疱疮病人头发作为对照。结果：35例大疱性类天疱疮患者的毛发,其中阳性28例,阴性7例,阳性率80%;类天疱疮IgG和C3在毛囊表皮下的外毛根鞘外层与结缔组织鞘之间呈明显的线状沉积。10例正常人及20例天疱疮患者均无线状沉积。结论：本实验方法简单、快速、敏感,且具有较高特异性,有可能成为诊断和鉴别诊断大疱性类天疱疮的一种有效的方法。     

上海复旦大学附属华山医院皮肤科办班消息

大疱性类天疱疮（RP）是一种慢性自身免疫性皮肤病，其主要的病理改变是表皮下水疱形成，免疫荧光检查示真表皮交界处C3和IgG等呈线状沉积，在患者血清中可检测到类天疱疮抗体，本研究拟通过检测大疱性类天疱疮患者拔出的头发上残留毛囊中类天疱疮抗体的沉积情况，以期建立一种快捷，损伤小的检测方法。     

大疱性类天疱疮患者头发毛囊中抗体检测的研究

大疱性类天疱疮(B P)是一种慢性自身免疫性皮肤病^[1],其主要的病理改变是表皮下水疱形成,免疫荧光检查示真表皮交界处C3和IgG等呈线状沉积,在患者血清中可检测到类天疱疮抗体。本研究拟通过检测大疱性类天疱疮患者拔出的头发上残留毛囊中类天疱疮抗体的沉积情况,以期建立一种快捷、损伤小的检测方法。     

天疱疮抗体在患者毛囊沉积的研究

天疱疮是一种大疱性皮肤黏膜疾病。在正常皮肤或黏膜上出现松弛性水疱,Nikolsky征阳性,血清中有天疱疮抗体。检测皮肤中天疱疮抗体的沉积,对指导临床治疗具有重要意义,但在临床上难以做反复的检查。本研究通过检测天疱疮患者拔出的头发上残留的毛囊中天疱疮抗体的沉积情况,建立一种简捷、损伤小的检测方法。     

天疱疮患者头发毛囊直接免疫荧光检测及其临床意义探讨

目的评价天疱疮患者头发毛囊直接免疫荧光检测的临床意义。方法每例患者头发约20根,采取直接染色方法进行直接免疫荧光检查。结果天疱疮患者毛囊直接免疫荧光示FITC标记的羊抗人IgG抗体在毛囊表皮下的外毛根鞘呈网状沉积,其荧光强度与天疱疮患者病情严重程度呈正相关(r=0.505,P0.05)。随访也发现,病情波动,毛囊DIF强度也随之变化,二者呈正相关。结论天疱疮患者毛囊直接免疫荧光强度在一定程度上反映与病情严重程度的相关性,推断对其强度的监测可用于评价疾病的严重程度并指导临床治疗。     

寻常性天疱疮的免疫学研究

一、天疱疮自身免疫的研究 1.荧光抗体法检查:1964年Beutner等报告,天疱疮患者血清中存在一种抗皮肤和可见粘膜上皮细胞间物质的自身抗体,在水疱、红斑等皮损处的表皮细胞间有IgG成网状沉积。此后人们提出,本病发生与自身免疫有关。1971年,Jordon等对为数众多的天疱疮患者进行免疫学检查,几乎全部病例     

大疱及疱疹性皮肤病

20051143 天疱疮抗体在患者毛囊沉积的研究/李晓东(中国医大附一院皮肤科),赵玉铭,王雅坤…//中华皮肤科杂志.-2004,37(10).-616-617 采用直接免疫荧光直接染色和切片两种方法检测6例天疱疮患者头皮毛囊天疱疮抗体的沉积情况。结果发现检测患者毛囊中自身抗体的沉积和患者的病变周围外观正常皮肤直接免疫荧光的结果一致,与类天     

湿疹中的循环基底膜带抗体

至少70％的全身性大疱性类天疱疮患者存在抗皮肤基底膜带(BMZ)的循环抗体,此抗体在正常人或其他大疱性皮肤病中不存在,除了妊娠疱疹及瘢痕性类天疱疮外,故对本病的诊断有重要的实际应用价值。作者报导用常规的免疫荧光技术处理,2例成人女性湿疹患者的皮损及其周围皮肤活检标本,其冷冻切片作直接免疫荧光检查,例1的BMZ处未检测到IgG;但7次检查中2次检测到有C_3,呈弱的线状型沉积。例2的BMZ处,2次检查无IgG,第3次检测到有很弱阳性的IgG呈线状型沉积;3次检查中1次查到C_3呈弱阳性线状型沉积。以正常人的皮肤、豚鼠唇、猴食道和病人自己的皮肤作为底物,作间接免疫荧光检查,发现2例患者的血清中含高滴度的抗BMZ的循环抗体,属IgG类抗体,免疫荧光的类型是线状型,与大疱性类天疱疮患者中所见的相同,未发现IgA或IgM类抗体。应用上述所     

天疱疮特异性抗体亚型与疾病活动的相关性研究

目的 探讨天疱疮患者抗桥粒芯糖蛋白（Dsg）1和Dsg3抗体亚型与疾病活动的相关性。方法 收集47例天疱疮患者血清,ELISA检测其特异性抗Dsg1和Dsg3抗体及亚型,分析抗体滴度及亚型与疾病活动的相关性。结果 抗Dsg1和Dsg3抗体类别与天疱疮临床类型有关,17例皮肤黏膜同时受累的患者中有14例（82.4%）同时存在两种抗体;16例仅皮肤受累的患者中有15例（93.7%）存在抗Dsg1抗体,仅1例（6.3%）存在抗Dsg3抗体;6例仅黏膜受累的患者只存在抗Dsg3抗体,阳性率为100％。随着病情的加重,抗Dsg1和Dsg3抗体滴度有上升趋势,但该趋势与疾病的严重度并不完全平行。而抗体亚型则和疾病活动相关,活动期以特异性IgG4亚型为主,稳定期以IgG1亚型为主。抗Dsg1阳性者,活动期特异性IgG4和IgG1抗体吸光度（A）值分别为1.92 ± 1.21和0.60 ± 0.61,IgG4/IgG1 > 1;稳定期特异性IgG4和IgG1抗体A值分别为0.03 ± 0.02和0.22 ± 0.11,IgG4/IgG1 < 1;抗Dsg3阳性者,活动期特异性IgG4和IgG1抗体A值分别为2.35 ± 2.17和1.84 ± 1.16,IgG4/IgG1 > 1;稳定期特异性IgG4和IgG1抗体A值分别为0.15 ± 0.16和1.05 ± 0.77,IgG4/IgG1 < 1。结论 天疱疮特异性抗体亚型与疾病活动密切相关,用ELISA检测天疱疮患者血清中抗Dsg1和Dsg3抗体类型、特异性抗体亚型及滴度,可更好地辅助诊断疾病、监测疾病活动。     

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.     

Immunofluorescence of the outer root sheath: an aid to diagnosis in pemphigus

Background. The direct immunofluorescence (DIF) pattern of the outer root sheath (ORS) of the hair follicle in patients with pemphigus resembles that of perilesional skin. We performed DIF on anagen and telogen hair ORS from patients with pemphigus and compared the findings with those of perilesional skin. Aim. To assess the sensitivity of DIF in the ORS of anagen and telogen hair in patients with pemphigus with positive DIF results in perilesional skin. Methods. In total, 50 patients with active pemphigus and positive DIF findings in perilesional skin were selected for the study. Anagen hair was obtained from the scalp by the same method used for a trichogram, and telogen hair was selected by combing the hair and collecting the loose strands of hair from the comb. The hair type was further confirmed by microscopy. Hair samples were transported in Michel medium and prepared for DIF by mounting on a slide without sectioning. Anagen and telogen hair samples were also collected from 50 controls. Results. Immunological deposits characteristic of pemphigus were found in the ORS of anagen and telogen hairs in all 50 patients, but in none of the 50 controls. Positive DIF findings were seen in scalp hair irrespective of the presence of scalp lesions. Of the 50 patients with pemphigus, 46 were diagnosed with pemphigus vulgaris, 2 with pemphigus vegetans, and 1 each with pemphigus foliaceus and pemphigus erythematosus, based on the clinical, histopathological and DIF findings. All the pemphigus variants had positive DIF findings in the ORS. Conclusions. Using DIF on telogen hair ORS promises to be a useful tool for diagnosis and follow‐up in cases of pemphigus.     

Th17相关细胞因子和趋化因子在天疱疮发病中的作用

目的：探讨Th17相关细胞因子IL-6、IL-17、IL-21、IL-22、IL-23和趋化因子CXCL-8、CCL-5在天疱疮发病中的作用。方法：采用酶联免疫吸附试验（ELISA）检测天疱疮患者治疗前后、大疱性类天疱疮患者及健康对照者的Th17相关细胞因子和趋化因子水平,并分析天疱疮严重程度及其亚型中这些因子的表达水平。结果：共收集天疱疮患者31例,其中寻常型天疱疮18例,落叶型天疱疮7例,红斑型天疱疮6例。另选取明确诊断为大疱性类天疱疮初发患者15例及20名健康人作为对照。天疱疮组中Th17相关因子IL-6、IL-17、IL-21、IL-22、IL-23和趋化因子CXCL-8、CCL-5水平分别为(32.31±7.98)pg/mL,(17.00±4.53)pg/mL,(491.13±72.41)pg/mL,(150.91±23.32)pg/mL,(264.89±67.84)pg/mL,(27.91±10.79)pg/mL,(47.21±17.83)pg/mL明显高于健康对照组的(8.36±1.96)pg/mL,(8.41±1.36)pg/mL,(372.73±18.45)pg/mL,(93.78±9.47)pg/mL,(163.17±9.48)pg/mL,(8.89±4.13)pg/mL,(22.66±2.85)pg/mL,差异均有统计学意义（P＜0.001）;天疱疮患者治疗后,IL-6、IL-17、IL-21、IL-22、IL-23和趋化因子CXCL-8水平较治疗前明显下降（P＜0.001）。IL-21在天疱疮组（491.13±72.41）pg/mL与大疱性类天疱疮组(559.58±78.56)pg/mL中差异有统计学意义（P＜0.05）,其余因子差异无统计学意义（均P＞0.05）;寻常型天疱疮、落叶型天疱疮及红斑型天疱疮患者的7种因子水平比较,差异均无统计学意义（均P＞0.05）;IL-17、IL-21、IL-22、IL-23、CCL-5与疾病严重程度有关（P＜0.05）。结论：Th17相关的细胞因子和趋化因子可能与天疱疮的发病和疾病严重程度有关。     

细胞涂片直接免疫荧光检查在天疱疮诊断上的应用

本文介绍了疱底细胞涂片直接免疫荧光检查的方法和75例水疱性皮肤病检查的结果.20例天疱疮涂片细胞表面可见特异性的荧光环,55例其它大疱性皮肤病的涂片均未见这种荧光.阐明了天疱疮细胞涂片直接免疫荧光阳性的机理,讨论了该法的临床应用价值,表明在不能作皮损活检冰冻切片直接免疫荧光检查时,该法是确诊天疱疮的一种简使有效的方法.     

Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases

Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.     

Indirect immunofluorescence testing in bullous diseases

Sera from patients with bullous pemphigoid, pemphigus vulgaris and other bullous conditions have been tested for auto-antibodies by the indirect immunofluorescence (IIF) method. Positive confirmation of the initial diagnosis was obtained in approximately 75% of both pemphigus and pemphigoid cases. It is suggested that the major source of error in the IIF test lies in the existence of ‘pemphigus-like antibodies’; and clinically, in diagnosing some cases of dermatitis herpetiformis as pemphigoid.     

天疱疮和大疱性类天疱疮患者血清瘦素水平的检测及意义

目的：探讨瘦素在天疱疮和大疱性类天疱疮发病中的作用。方法：采用放射免疫分析法对35例天疱疮和大疱性类天疱疮患者血清瘦素水平进行了检测，并以30例健康体检者作为正常对照组。结果：天疱疮和大疱性类天疱疮患者瘦素水平与正常对照组相比，显著升高，差异有显著性（P〈0．001）；重症和中、轻症患者瘦素水平升高，与正常对照组相比，差异均有显著性（P〈0．01和P〈0．05）；重症与中、轻症患者相比较，瘦素水平差异无显著性（P〉0．05）。男、女天疱疮和大疱性类天疱疮患者瘦素水平与正常对照组相比均明显升高，差异有显著性（P〈0．005和P〈0．01）；男及女天疱疮、大疱性类天疱疮患者瘦素水平较其各自正常对照组升高，差异有显著性（P〈0．05和P〈0．01）。结论：天疱疮和大疱性类天疱疮患者瘦素水平升高，可能在这两种大疱性疾病的发生和发展过程中起一定作用。     

Reliability of cytodiagnosis in oral pemphigus vulgaris. A study of 30 cases

The aim of this study was to verify the reliability of the Tzanck test, performed by both traditional cytomorphology and the direct immunofluorescence technique, in diagnosis of oral pemphigus vulgaris. Cytologic smears were obtained from oral erosions of thirty patients with well ascertained pemphigus vulgaris. Control smears were taken from thirty patients affected by various other diseases of oral mucosa and from thirty healthy subjects. Cytologic diagnosis of oral pemphigus was based upon significant findings of acantholytic cells by cytomorphology, and of epithelial cells with pericellular deposition of IgG (which persisted after cytocentrifugation) by direct immunofluorescence. The cytomorphological test gave positive results in twenty-eight patients with pemphigus and in one patient with herpetic stomatitis, and negative results in all other cases. The immunocytologic test gave positive results in all patients with pemphigus, and negative results in all controls. These findings indicate that the cytomorphological test may be a useful method for screening oral pemphigus, while the immunocytologic test may provide a reliable method for definitive diagnosis.     

Specific detection of anti-cell surface antibodies in herpes gestationis sera

Abstract We examined 42 herpes gestationis sera with immunofluorescence of normal human skin sections, and found that anti-keratinocyte cell surface antibodies were detected specifically in 10 herpes gestationis sera. The diagnosis of these herpes gestationis cases was confirmed by detecting antibodies against the 180 kD bullous pemphigoid antigen with immunoblotting of its fusion protein. The results of immunoadsorption assay using baculoproteins of both pemphigus vulgaris and pemphigus foliaceus antigens indicated that the herpes gestalionis sera did not recognize common pemphigus antigens. Immunoblotting of human epidermal extracts and immunofluorescence of various tissues also suggested that the sera did not recognize any other desmosomal components or paraneoplastic pemphigus antigens. The significance of this reactivity is unclear. However, because no control bullous pemphigoid sera showed this reactivity, it may suggest a different pathophysiology between herpes gestationis and bullous pemphigoid.     

Antidesmoglein autoantibodies in silicosis patients with no bullous diseases

BACKGROUND: Pemphigus is an autoimmune bullous disease characterized by the presence of antidesmoglein autoantibodies. However, the mechanism of its autoantibody production remains unknown. In previous reports, we have described rare cases of pemphigus and pemphigoid associated with silicosis. It is well known that during long-term silicosis, some autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus or rheumatoid arthritis, can occur. OBJECTIVE: The aim of this study was to explore the presence of pemphigus or pemphigoid autoantibodies in silicosis patients without clinical bullous diseases or collagen diseases. METHOD: The presence of pemphigus antibodies was examined in 54 silicosis patients with no associated bullous diseases, using immunofluorescence, the enzyme-linked immunosorbent assay (ELISA) for desmoglein 1 and 3, and immunoblotting methods. In the antibody-positive cases, HLA genotyping of peripheral lymphocytes was performed with PCR-RFLP. RESULTS: Seven out of the 54 patients were found to be positive for pemphigus antibodies and 1 for bullous pemphigoid by immunofluorescence. In addition, by ELISA, 6 patients were found to be positive against the desmoglein 1 antigen, 2 against the desmoglein 3 antigen and 2 against both desmoglein 1 and desmoglein 3. CONCLUSION: The results of the present study strongly suggest the occurrence of pemphigus and pemphigoid autoantibodies in patients with silicosis. It remains unclear whether such patients will develop an autoimmune bullous disease in the future. Accordingly, long-term follow-up of antibody-positive patients is required.