C3d在大疱性类天疱疮患者皮损组织中的表达

目的 探讨C3d在石蜡包埋的大疱性类天疱疮患者皮损组织中的表达及临床意义。方法 免疫组化SP法在25例大疱性类天疱疮、10例大疱性表皮松解症及10例正常成人皮肤组织标本中进行C3d、IgG、IgA进行检测,并对其在大疱性类天疱疮皮损中阳性率进行比较。结果 大疱性类天疱疮皮损中C3d、IgG、IgA的阳性率分别为96%、72%、0%。C3d、IgG在BP组织中表达阳性率的差异有统计学意义（χ2 = 4.17,P < 0.05）,C3d、IgA在BP组织中表达阳性率的差异有统计学意义（χ2 = 22.04,P < 0.01）。C3d、IgG、IgA在10例EB及正常成人皮肤组织标本中表达均为阴性。结论 免疫组化方法检测C3d的表达可以协助在石蜡组织中进行大疱性类天疱疮的诊断。     

表现为环状红斑水疱的自身免疫性表皮下水疱病25例回顾性分析

目的总结表现为环状红斑水疱的自身免疫性表皮下水疱病的临床、组织病理、免疫血清学及治疗特点。方法回顾性分析2015—2022年就诊于中国医学科学院皮肤病医院表现为环状红斑水疱的自身免疫性表皮下水疱病患者的资料。结果共纳入患者25例, 男10例、女15例, 年龄(39.21 ± 24.65)岁, 包括线状IgA大疱性皮病9例, 大疱性类天疱疮7例, 抗P200类天疱疮5例, 获得性大疱性表皮松解症4例, 20例(80%)有不同程度瘙痒。15例(60%)出现真皮组织嗜酸性粒细胞浸润, 11例(44%)外周血嗜酸性粒细胞计数增加, 7例(28%)同时有嗜酸性粒细胞组织浸润和外周血嗜酸性粒细胞升高。盐裂皮肤-间接免疫荧光及免疫印迹实验显示, 9例同时存在抗基底膜带IgG及IgA抗体, 包括4例大疱性类天疱疮、1例线状IgA大疱性皮病、2例抗P200类天疱疮、2例获得性大疱性表皮松解症;5例同时存在多种抗基底膜带靶抗原的抗体。7例大疱性类天疱疮均予系统糖皮质激素治疗, 其中5例联合免疫抑制剂, 2例联合米诺环素;线状IgA大疱性皮病、抗P200类天疱疮、获得性大疱性表皮松解症患者对抗炎药物及氨苯砜...     

类天疱疮样扁平苔藓1例

目的：报告和分析一例少见的类天疱疮样扁平苔藓病例。方法：取患背部皮损活检组织部分行组织病理和免疫病理检查。结果：活检组织切片,HE染色示：表皮角化过度伴角化不全,颗粒层不规则增厚,基底细胞液化严重已融合成表皮下大疱,无大疱区域表皮和真皮分界不清。有较多淋巴细胞在此区域以及血管周浸润,可见“色素失禁”现象。直接免疫荧光显示基底膜有IgG、C3、IgM呈线状沉积。符合类天疱疮样扁平苔藓。经治疗后痊愈,随访一年未见复发。结论：类天疱疮样扁平苔藓临床表现和病理结果不同于大疱性扁平苔藓和大疱性类天疱疮,是一个独立的疾病。可采用灰黄霉素、四环素和烟酰胺治疗。     

泛发性萎缩性良性大疱性表皮松解症我国首例报道

目的 报道我国首例泛发性萎缩性良性大疱性表皮松解症家系。方法 对该家系先证者的临床资料、组织病理、透射电镜、间接免疫荧光检查进行分析。结果 该患者除了先天性大疱性表皮松解症的症状外,特征性表现是萎缩性秃发和牙齿发育不良。透射电镜检查裂隙位于基底膜透明板,同时伴半桥粒数目减少和发育不良。间接免疫荧光检查发现患者针对大疱性类天疱疮抗原2的荧光消失,说明本例发病与编码大疱性类天疱疮抗原2的基因COL17A1的缺陷有关。结论 此例为泛发性萎缩性良性大疱性表皮松解症,是交界性大疱性表皮松解症的一种特殊亚型。泛发性萎缩性良性大疱性表皮松解症有一定的临床特点,透射电镜和间接免疫荧光检查对于正确诊断和分型十分重要,并对进一步基因突变位点研究有指导作用。     

大疱及疱疹性皮肤病

20 0 0 32 4 5　自身免疫性表皮下大疱病基底膜带自身抗体的检测 /杨森 (安徽医大一附院皮肤科 )…∥中国皮肤性病学杂志 .- 2 0 0 0 ,14( 3) .- 145分别应用盐裂皮肤间接免疫荧光 ( IIF)及比较免疫印迹 ( IB)技术对 97例自身免疫性表皮下大疱病( SABD)基底膜带自身抗体 ( BMZ- Ab) Ig G型或 Ig A型进行检测。 97例 SABD中大疱性类天疱疮 ( BP) 63例 ,线性 Ig A大疱病 ( L AD) 2 2例 ,获得性大疱性表皮松解症 ( EBA) 6例 ,大疱性 SLE( BSL E) 6例 ,10例夏季皮炎患者血清作对照。结果 :IIF检查 SABD中 Ig G型或 Ig A型 B…     

天疱疮大疱性类天疱疮病损组织中TNFα TNFRp55TNFRp75和iNOS的免疫组化检测

目的探讨TNFα、TNFRp55、TNFRp75、诱生型一氧化氮合成酶(iNOS)在天疱疮、大疱性类夫疱疮病损组织中所起的作用.方法应用免疫组化技术,对14例天疱疮、11例大疱性类天疱疮的皮损进行了检测.结果显示TNFα、TNFRp55、TNFRp75、iNOD在天疱疮和大疱性类天疱疮皮损中均有不同程度的表达,尤其在水疱的顶、底部表达更明显.结论TNFα通过与其受体TNFRp55和TNFRp75结合,在天疱疮表皮角质形成细胞的凋亡和水疱形成、大疱性类天疱疮基底膜带的破坏中可能起一定作用;TNFα可诱导细胞高表达jNOS,产生过量的一氧化氮(NO),可能引起组织的损伤,也可能是加剧天疱疮和大疱性类天疱疮组织损伤的一种因素.     

儿童大疱性类天疱疮1例

报告1例儿童大疱性类天疱疮,患儿女,8岁,2月前躯干、四肢皮肤出现红斑、水疱、大疱,尼氏征阴性。皮损组织病理检查示:表皮下水疱,疱腔内有嗜酸性粒细胞、中性粒细胞浸润;直接免疫荧光示:IgG、C3线状沉积于基底膜带。诊断为儿童大疱性类天疱疮,静注甲强龙治疗后效果良好,随访至今未复发。     

放射性皮炎继发大疱性类天疱疮一例

【摘要】 目的 报告放射性皮炎继发大疱性类天疱疮1例,探讨大疱性类天疱疮与放射性皮炎的相关发病机制。病例内容 患者女,82岁,左乳腺癌切除术后10年,术后放疗处出现放射性皮炎。半月前于放射性皮炎位置开始出现浮肿性红斑,水疱,大疱,尼氏征阴性,并伴有瘙痒,随后于背部,下肢出现二处水疱。组织病理为表皮下疱,免疫病理显示基底膜带IgG和C3沉积,血清中存在针对基底膜带成分的自身抗体。予米诺环素加烟酰胺治疗两周后病情控制满意。结论 放射性皮炎继发大疱性类天疱疮国际上偶见报道,有学者认为局部放疗能改变基底膜的性质,使自身抗原暴露,引起自身免疫反应;并能影响金属基质蛋白酶和血管内皮生长因子的表达,故认为大疱性类天疱疮有可能是放疗的一个潜在副作用。     

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。     

中药联合免疫抑制剂治疗大疱性类天疱疮1例

大疱性类天疱疮是自身免疫性大疱病，出现表皮下大疱，基底膜带有免疫球蛋白和补体沉积，多数患者血清中有抗表皮基底膜带自身抗体。治疗上按轻重程度的不同采用不同的治疗方案，但重症病人治疗较为困难，尤伴有其它基础疾病的病人，而我们在临床上运用中药辨证联合免疫抑制剂成功的治疗了1例重症大疱性类天疱疮病人，现报道如下。     

C4d immunohistochemical stain is a sensitive method to confirm immunoreactant deposition in formalin-fixed paraffin-embedded tissue in bullous pemphigoid

Background:  Bullous pemphigoid (BP) is characterized clinically by the onset of pruritic urticarial plaques, vesicles and bullae in a predominantly elderly population. While the diagnosis may be suspected on routine hematoxylin and eosin histology of formalin-fixed paraffin-embedded tissue, fresh-frozen tissue must be used to show the immunologic nature of the bullous process by direct immunofluorescence (DIF). The diagnosis is further confirmed and separated from epidermolysis bullosa acquisita (EBA) by subsequent serologic studies to detect antibodies directed against BP180 and BP230 antigens and characteristic antibody deposition on salt-split skin.
Methods:  Using a polyclonal complement fragment 4d (C4d) antibody, we stained formalin-fixed paraffin-embedded skin biopsy specimens from cases of BP and controls.
Results:  We showed characteristic linear basement membrane deposition of C4d in formalin-fixed paraffin-embedded tissue in seven of nine cases diagnosed as BP vs. EBA by DIF on fresh-frozen tissue. None of the four controls for which we had adequate tissue were positive.
Conclusion:  These results indicate that formalin-fixed paraffin-embedded tissue can be stained for the immunoreactant C4d to show characteristic immunoreactant deposition, potentially obviating the need for repeat biopsy for DIF and allowing clinicians to proceed to serologic confirmation of BP.     

C3d immunohistochemistry on formalin‐fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin

Background: Direct immunofluorescence (DIF) testing is an important procedure in the diagnosis of autoimmune bullous dermatoses. We investigated the expression of C3d in formalin‐fixed, paraffin‐embedded tissue of autoimmune bullous dermatoses. Methods: The immunohistochemical expression of C3d in bullous pemphigoid (BP) (n = 32), pemphigoid gestationis (PG) (n = 3), pemphigus (n = 14), dermatitis herpetiformis Duhring (DHD) (n = 10), linear immunoglobulin A (IgA) dermatosis (n = 4), mixed forms of BP and linear IgA dermatosis (n = 2), and 44 controls was analyzed on formalin‐fixed tissue. Results: Thirty‐one of 32 cases (97%) of BP and 3 out of 3 cases (100%) of PG showed a linear positivity of C3d along the basement membrane. Only 3 out of 14 (21%) cases of pemphigus showed an intraepidermal intercellular expression of C3d. The two mixed forms of linear IgA dermatosis and BP showed a linear positivity of C3d along the basement membrane. All cases of DHD, linear IgA dermatosis and all of the controls were negative for C3d. Conclusions: C3d immunohistochemistry is a valuable tool in the diagnosis of BP and PG of the skin with a sensitivity of at least 97%. Mixed forms of linear IgA dermatosis, and BP, DHD and linear IgA dermatosis can only be identified by DIF. A positive result may prompt serologic confirmation of BP without further need for DIF. Pfaltz K, Mertz K, Rose C, Scheidegger P, Pfaltz M, Kempf W. C3d immunohistochemistry on formalin‐fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin.     

The generalized atrophic benign form of junctional epidermolysis bullosa. Experience with four patients in the United States

We encountered four patients in the United States with the generalized atrophic benign form of junctional epidermolysis bullosa (epidermolysis bullosa atrophicans generalisata mitis, nonlethal junctional epidermolysis bullosa). Prior to the performance of definitive diagnostic studies, each patient had been thought for at least a decade to have either a dystrophic or simplex form of epidermolysis bullosa. Each patient had generalized blisters since birth that healed with atrophy and mild scarring but without milia or contractures. Two of the four patients had experienced laryngeal involvement during childhood. In each patient, correct diagnosis was finally established by either electron microscopic examination or immunofluorescence mapping of skin sections from induced blisters.     

Location of basement membrane Type IV collagen beneath subepidermal bullous diseases

Using standard immunohistochemical methods, routinely processed sections, and a polyclonal antibody to Type IV collagen, we have determined the location of Type IV collagen, a substance located in the lamina densa of basement membrane, in a spectrum of acquired subepidermal bullous diseases. Type IV collagen was attached to the blister roof in five cases of well-established epidermolysis bullosa acquisita and to the blister base in 25 cases of bullous pemphigoid, four cases of dermatitis herpetiformis and 12 cases of porphyria cutanea tarda. Immunohistochemical localization of Type IV collagen in epidermal-dermal basement membrane is a simple, rapid and reliable technique which can be utilized to exclude and possibly to confirm the diagnosis of epidermolysis bullosa acquisita in routinely fixed paraffin-embedded tissues.     

Defective expression of basement membrane-associated C3d,g in papulonodular basal cell carcinomas

Recent studies in our laboratory have shown that C3d,g, a 41,000-Da fragment of the third component of complement, is present along the base of the lamina densa and in the sublamina densa region of normal human epidermal basement membrane, but absent from the skin of a patient with congenital C3 deficiency. In studies of human skin, papulonodular basal cell carcinomas have served as a useful model for the investigation of various basement membrane antigens and matrix proteins. To further investigate the presence of C3d,g within epidermal basement membrane as well as examine its relationship with other known basement membrane constituents, we have analyzed serial sections of ten papulonodular basal cell carcinomas by light and immunofluorescence microscopy. In these studies, C3d,g was either absent (N = 9) or minimumly detectable (N = 1) in tumor nest basement membranes. While bullous pemphigoid and KF-1 antigens were absent (N = 6 and N = 3, respectively) or significantly decreased (N = 4 and N = 7, respectively), epidermolysis bullosa acquisita antigen was routinely present though somewhat (N = 3) or moderately decreased (N = 3). Laminin and type IV collagen were expressed normally in all tumor nest basement membranes. All constituents, including C3d,g, were present in adjacent normal epidermal basement membrane of these tumor samples. This study has demonstrated antigenic alterations within each ultrastructural subregion of papulonodular basal cell carcinoma tumor nest basement membranes by identifying the virtual absence of C3d,g (sublamina densa) as well as a significant reduction in KF-1 (lamina densa) and bullous pemphigoid (lamina lucida) antigens. Moreover, the presence of laminin, type IV collagen, and epidermolysis bullosa acquisita antigen in tumor nest basement membranes suggests that these particular constituents neither cleave C3 nor act as essential binding sites for passive incorporation of this complement component in epidermal basement membrane. These studies give additional support to the hypothesis that C3d,g is a previously unrecognized constituent of normal epidermal basement membrane and does not represent passive incorporation of circulating C3 at this site in human skin.     

A comparative study of immunohistochemistry and electron microscopy used in the diagnosis of epidermolysis bullosa

Electron microscopic examination still is the gold standard for classifying epidermolysis bullosa, although it is relatively expensive, time consuming, and not readily available. Immunoreagents have been developed recently to map antigens in the basement membrane on routinely processed specimens. The current study was performed to examine the diagnostic usefulness of immunohistochemistry, as compared with electron microscopic examination, for analyzing routine formalin-fixed paraffin-embedded sections of epidermolysis bullosa. This study investigated 39 consecutively diagnosed cases of epidermolysis bullosa in which both electron microscopic examination and immunohistochemistry were used. In each case, three monoclonal antibodies were used to stain for laminin 1, collagen IV, and keratin. The immunohistochemical patterns were defined as follows: epidermolysis bullosa simplex (laminin, collagen IV, or both at the dermal floor of the blister and keratin at both the dermal floor and the epidermal roof), junctional epidermolysis bullosa (laminin, collagen IV, or both at the dermal floor of the blister and keratin only at the epidermal roof), and dystrophic epidermolysis bullosa (collagen IV, laminin, or both, and keratin all at the epidermal roof). Altogether, electron microscopic examination subclassified epidermolysis bullosa into its three major forms in 37 of the 39 cases (95%), and immunohistochemistry in 33 of the 39 cases (85%). All of the classifiable cases were concordant. Specifically, immunohistochemistry was diagnostic in 10 of 14 (71%) epidermolysis bullosa simplex cases, 14 of 14 (100%) junctional epidermolysis bullosa cases, and 9 of 11 (82%) dystrophic epidermolysis bullosa cases. The most frequent cause for inconclusive immunohistochemical results was failure in staining of the basement membrane with the antibodies to both laminin and collagen IV. In conclusion, the use of immunohistochemistry on routinely processed specimens may be useful for subclassifying epidermolysis bullosa into its major forms in the majority of the cases, although it still cannot fully replace electron microscopic examination or immunofluorescence mapping in the diagnosis of epidermolysis bullosa.     

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。     

Schleimhautbeteiligung bei blasenbildenden Erkrankungen

Chronic involvement of orogenital and conjunctival mucosa in the course of either genetically based (epidermolysis bullosa hereditaria) or auto-immunologically mediated (as for example pemphigus vulgaris, mucous membrane pemphigoid or epidermolysis bullosa acquisita) blistering diseases can cause significant morbidity. To provide accurate care, recognition of clinical, pathogenic and diagnostic features as well as awareness of recent advances in the development of new therapeutic modalities are mandatory and thus will be discussed in this review.