大疱及疱疹性皮肤病

961705 皮肤直接免疫荧光检查在大疱性皮肤病中的诊断意义/毛舒和…//中国皮肤性病学杂志。-1996,10(2).-109 对87例大疱性皮肤病的皮肤直接免疫荧光检查(DIF)和病理学检查进行了对比分析,结果DIF诊断阳性率显著高于病理学检查阳性率(P<0.05)。本组中临床、病理和DIF诊断三者一致的有42例,占48.2％;有一半以上三者不一致,因此单凭临床和病理学检查难以确诊,必须借助皮肤DIF检查才能确诊。DIF技术在大疱性皮肤病的诊断上较病理学检查更具有诊断意义。表1参2     

大疱及疱疹性皮肤病

951737 盐裂后直接免疫荧光检测用于大疱性皮肤病诊断/翁孟武…//上海医科大学学报。-1995,22(2).-148 对30例大疱性皮肤病应用1mol/L NaC1盐裂皮损皮肤作直接免疫荧光检查(盐裂DIF),并与IIF、盐裂IIF和免疫印迹结果比较。结果:除1例家慢良外,     

大疱性皮肤病直接免疫荧光检查结果分析

大疱性皮肤病是一组与自身免疫密切相关的疾病,单纯依靠临床或常规组织病理往往难以确诊,常常需借助于免疫荧光技术.现将近两年来我科对大疱性皮肤病开展直接免疫荧光检查(DIF)的结果及临床意义分析如下.     

80例大疱性皮肤病组织病理及直接免疫荧光结果分析

目的：探讨组织病理及直接免疫荧光检查对大疱性皮肤病的诊断意义。方法：对80例大疱性皮肤病患者的皮损进行组织病理检查,并采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,对结果进行回顾性分析。结果：本组患者自身免疫性大疱性皮肤病占60.00%,以天疱疮和类天疱疮为主,非自身免疫性大疱性皮肤病以大疱性表皮松解症及大疱性多形红斑多见,不同类型的大疱性皮肤病组织病理及免疫荧光具有特征性。结论：组织病理及直接免疫荧光检查对大疱性皮肤病的诊断、鉴别诊断、治疗、预后判断具有重要的意义。     

大疱性疾病正常皮肤免疫荧光检测的临床意义

本文DIF检测天疱疮正常皮肤阳性率为93.2%,大疱性类天疱疮正常皮肤阳性率为100%,因此作者认为进行正常皮肤的DIF检测对无皮肤损害的天疱疮、大疱性类天疱疮的早期诊断、鉴别诊断、推测予后具有重要意义。     

1M NaCl分离皮肤的免疫荧光法在表皮下大疱病诊断中的应用

该文介绍1M NaCl分离皮肤免疫荧光法诊断表皮下大疱病的原理和优越性。同时介绍了盐裂正常人皮肤IIF法以及盐裂皮损周围皮肤的DIF法对表皮下大疱病诊断与鉴别诊断的价值。     

143例天疱疮、大疱性类天疱疮直接免疫荧光检查结果分析

目的回顾性观察天疱疮、大疱性类天疱疮直接免疫荧光检查(DIF)结果,分析及评估其实验方法。方法活检取材,冰冻切片,DIF染色,荧光显微镜观察。结果免疫病理确诊143例,其中寻常型天疱疮(PV)阳性75例(52.4%)、红斑型天疱疮(PE)阳性13例(9.1%)、大疱性类天疱疮(BP)阳性53例(37.1%)、副肿瘤天疱疮(PNP)2例(1.4%)。临床与免疫病理诊断的符合率PV为65.3%(49/75),PE为61.5%(8/13),BP为69.8%(37/53),三者比较差异无统计学意义(P>0.05)。结论DIF对常见大疱性皮肤病的诊断是一项重要的、快速而特异性较高的检查方法,但仍需紧密结合临床确诊。     

免疫荧光技术在诊断大疱性皮肤病中的应用

免疫荧光(IF)技术对诊断大疱性皮病有相当大的帮助,但它不能代替常规的皮肤组织病理检查,只有将这两种方法和临床检查结果结合起来,才能避免作出错误的诊断.检查方法(一)直接免疫荧光技术(DIF)皮肤标本:最好取自早期水疱或大疱边缘部位的皮肤.一般来说不宜用钻孔法,它只能用于疱疹样皮炎(DH)外观正常皮肤的取材.目前多用快速冷冻法来固定标本.对于尚无条件作免疫病理检查的医院,可用     

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     

盐裂皮肤间接免疫荧光及BP180 NC16a酶联免疫吸附测定在大疱性类天疱疮诊断中的意义

目的 探讨盐裂皮肤间接免疫荧光及大疱性类天疱疮（BP）180 NC16a-酶联免疫吸附测定（ELISA）检测在BP诊断中的意义。方法 收集2015年1月至2017年8月在中国医学科学院皮肤病医院用盐裂皮肤间接免疫荧光（IIF?SSS）和BP180 NC16a?ELISA检测BP患者174例和对照组129例血清。其中25例BP患者用直接免疫荧光（DIF）进行检测并与IIF?SSS和BP180 NC16a?ELISA敏感性进行比较。结果 IIF?SSS、BP180 NC16a?ELISA的敏感性分别为93.67%、96.55%;特异性分别为100%、96.12%。IIF?SSS与BP180 NC16a?ELISA相关系数0.147,为弱相关。其中25例BP患者血清学诊断方法（IIF?SSS,BP180 NC16a?ELISA）和DIF敏感性比较差异无统计学意义。结论 BP血清学诊断方法特异性强、敏感性高,值得临床推广应用。     

Repeat direct immunofluorescence (DIF) test, using, 1 M NaCl treated skin, in the subepidermal autoimmune bullous diseases that contain IgG at the dermal epidermal junction

Knowledge of autoimmune bullous diseases has greatly increased with the recognition of new entities, and the use of the direct immunofluorescence (DIF) using 1 molar per liter of sodium chloride (1 M NaCl) treated skin has been proposed. To estimate the frequency with which the different DIF patterns are present, we performed a systematic study of the skin or oral mucosa samples in which linear deposits of IgG at the basement membrane zone were detected by routine DIF in the last 6 years. The DIF tests were done on 56 samples before and after splitting the epidermis from the dermis with 1M NaCl. In 40 biopsies (72%) IgG was found on either the epidermal side or on both sides after 1M NaCl split. These cases corresponded to bullous pemphigoid (n=33), herpes gestationis (n=5) and cicatricial pemphigoid (n=2). In 6 cases (10.7%), IgG deposits were observed only on the floor, five corresponding to bullous pemphigoid and one to bullous pemphigoid-like eruption induced by amoxicillin. Repeat direct immunofluorescence using 1M NaCl split skin indicates that at least 12% of patients who were initially diagnosed as bullous pemphigoid, may in fact suffer a different entity, requiring other techniques to achieve the right diagnosis. This test can be a useful routine screening for autoimmune bullous diseases.     

表皮下大疱病的鉴别诊断和抗原表达区域性差别的研究

通过间接免疫荧光和盐裂皮损周围皮肤直接免疫荧光（简称盐裂ＤＩＦ），分别研究正常人皮肤、类天疱疮（ＢＰ）及获得性大疱性表皮松解症（ＥＢＡ）抗原表达的区域性差别和表皮下大疱病鉴别诊断。 窝、肘窝、上背、下背、股内侧和下腹部皮肤ＢＰ抗原表达率较高；膝、阳窝、足背、肘、肘窝和下腹部皮肤ＥＢＡ抗原表达率较高。皮肤ＤＩＦ显示２５例表皮下大疱病中１６例（６４％）基底膜带有Ｃ３或ＩｇＧ或伴Ｃ３和ＩｇＡ沉积；盐裂ＤＩＦ表明２５例（１００％）均有ＩｇＧ或伴Ｃ３和ＩｇＡ沉积在表皮侧或真皮侧。结果提示，ＢＰ抗原高表达率与皮损好发部位相一致；ＥＢＡ抗原高表达率一部分与皮损好发部位一致。盐裂ＤＩＦ不仅提高ＤＩＦ阳性率，而且根据免疫反应物沉积部位可以鉴别出ＢＰ与ＥＢＡ以及大疱性系统性红斑狼疮。     

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。     

Clinical, histopathologic, and therapeutic aspects of subepidermal autoimmune bullous diseases with IgG on the floor of salt-split skin

BACKGROUND: About 12% of patients with subepidermal autoimmune bullous disease and immunoglobulin G (IgG) at the dermal-epidermal junction present diseases other than bullous pemphigoid. MATERIALS AND METHODS: We report the clinical, histopathologic, and therapeutic aspects of eight cases of subepidermal bullous disorder with IgG on the floor of salt-split skin. RESULTS: A predominant neutrophilic infiltrate was detected in six of the eight patients. In one patient, the inflammatory infiltrate was neutrophilic and eosinophilic in the same proportion. A good response to dapsone alone or combined with prednisone was observed in six patients. CONCLUSIONS: The salt-split skin direct immunofluorescence test is useful for its diagnostic and therapeutic implications.     

病情与外周血嗜酸粒细胞计数平行的大疱性类天疱疮一例

报告1例病情与外周血嗜酸粒细胞计数相平行的大疱性类天疱疮.患者男,65岁.全身泛发红斑、水疱1个月,加重15天.皮肤科检查:皮损泛发头面颈、躯干、四肢及会阴部,基本损害为正常皮肤或红斑基础上出现大小不等的水疱,疱液为草黄色,散在红色糜烂面,尼氏征阴性,部分皮损表面敷以灰褐色痂片,以胸背部、四肢尤为严重.组织病理、直接免疫荧光、间接免疫荧光及盐裂皮肤直接免疫荧光检查结果符合大疱性类天疱疮.入院后依据病情变化,先后给予3次糖皮质激素冲击治疗,治疗过程中,定期监测血常规,提示外周血嗜酸粒细胞计数随病情波动.     

Immunofluorescence Study of Pemphigus from North India

Both serum studies by indirect immunofluorescence (IIF) tests and skin biopsy examination by direct immunofluorescence (DIF) were performed on 22 cases of pemphigus with clinically active lesions. Twenty cases had pemphigus vulgaris and two, pemphigus foliaceus. The majority of cases (70%) were between 21 and 60 years old; the mean age was 39.5 ± 12.7 years. There was no sex predilection. DIF showed the positive fluorescence of intercellular cement substance (ICS) of the epidermis in all 22 cases (100%). IgG was positive in 77.27%, followed sequentially by C3C (50%), IgA (45.45%), and IgM (36.36%). Six cases (27.27%) also showed granular immunoglobulin and/or complement deposits at the dermoepidermal junction. IIF detected serum antiepithelial antibodies in 18 cases (81.81%) using human esophagus as substrate. Most of these cases (88.88%) showed IgG type of antibodies; the other 11.11% exhibited IgA and IgM in low concentrations. 1+ titer positivity was observed in 15 cases. This study demonstrates the value of DIF for a definitive diagnosis of pemphigus. However, it is also important to appreciate that immunofluorescence is not a substitute for histopathology, but rather complementary to it.     

Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo–epidermal junction

Cutaneous mastocytosis is a disease characterized by the infiltration and proliferation of mast cells in the skin. In children, the most common form of presentation is urticaria pigmentosa, while the diffuse cutaneous bullous mastocytosis is one of the rarest subtypes seen. The aim of this paper is to present a case of diffuse bullous mastocytosis with detection of IgM deposits at dermo–epidermal junction using direct immunofluorescence (DIF) microscopy. The diagnosis of diffuse bullous mastocytosis is a challenge, and DIF microscopy is necessary in order to exclude an autoimmune bullous disorder. However, IgM deposits at dermo–epidermal junction can be nonspecific, being found in a variety of skin disorders. A 6‐month‐old girl presented with bullous lesions and erosions on the scalp and the trunk. During hospitalization, further bullous lesions appeared, along with generalized erythrodermia. Skin biopsy revealed aspects of urticaria pigmentosa. Taking into account the clinical findings, the case was enclosed as bullous mastocytosis. Treatment included the avoidance of trigger factors, and administration of antihistamines along with a short‐term course of systemic steroids. The evolution was favorable, with remission of the existing lesions and without occurrence of new ones.     

CUTANEOUS LUPUS ERYTHEMATOSUS: COMPARISON OF DIRECT IMMUNOFLUORESCENCE FINDINGS WITH HISTOPATHOLOGY

Background. Direct immunofluorescence (DIF) is considered to be a major advance in the diagnosis of connective tissue diseases, particularly lupus erythematosus (LE); however, the reliability of the technique depends on several factors, such as age and site of the lesion, type of immunofluorescence, type of immunoglobulin, etc. False positives and false negatives can occur. Objective and Methods. To determine the diagnostic value of DIF we studied 18 clinically established cases of cutaneous lupus erythematosus (CLE). Lesional biopsies were subjected to routine histopathologic examination and direct immunofluorescence. The results were compared. Results. Direct immunofluorescence was positive in 72.7% and histopathology in 66% cases. Combination of the two techniques (with one or both methods giving characteristic findings) was positive in 83% cases. The most common antibody was IgG, seen in 77.8% cases. A homogeneous pattern of immunofluorescence, with IgG, was seen in 55.5% of the cases. Although histopathology gave positive or suggestive results in all cases, DIF was negative in two cases of early cutaneous LE. Conclusion. Although DIF is an extremely useful diagnostic tool, it should always be used in conjunction with histopathology and the combination of the two methods yields the best results.     

Frequency of IgA antibodies in pemphigus, bullous pemphigoid and mucous membrane pemphigoid

Circulating and bound IgA antibodies can be found in the autoimmune blistering diseases, but their prevalence, clinical relevance and target antigens remain unknown. Thirty-two patients with pemphigus, 73 with bullous pemphigoid and 28 with mucous membrane pemphigoid were studied retrospectively. Direct immunofluorescence (DIF) analysis of IgG, IgA, IgM and C3 was carried out for all cases. Sera were studied by standard indirect immunofluorescence, indirect immunofluorescence on salt-split skin, immunoblotting for bullous pemphigoid and mucous membrane pemphigoid and ELISA for pemphigus. With DIF, we found IgA autoantibodies in 22 of all 133 cases. Circulating IgA antibodies to skin were detected in 2 of 3 IgA-DIF-positive patients with pemphigus, in 3 of 6 with bullous pemphigoid, and in 6 of 13 with mucous membrane pemphigoid. We confirm that the IgA reactivity is more frequently associated with mucous membrane involvement, especially in cases without critical involvement (5/8). The role of IgA and its antigenic specificity in these diseases remain unclear.