天疱疮患者头发毛囊与皮损中自身抗体沉积的比较

目的通过检测天疱疮患者拔出的头发上残留毛囊和皮损中天疱疮抗体的沉积情况,建立一种简便、迅速的检测方法。方法直接免疫荧光方法测定21例天疱疮患者拔出的头发上残留毛囊中和皮损处天疱疮抗体的沉积。结果FITC标记的IgG抗体在19例天疱疮患者毛囊外毛根鞘的1～2层呈明显连续的网状或珠状沉积,2例稳定期天疱疮患者为阴性。在检测的19例天疱疮患者中17例皮损表皮内棘细胞间IgG呈网状沉积,2例稳定期患者为阴性。在8例大疱性类天疱疮患者及10例正常人无网状沉积。结论检查毛囊处天疱疮抗体沉积的实验方法简便、快速,无须切片,与皮损检查结果一致,有可能成为天疱疮诊断的一种常规检测方法。     

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。     

IgA型基底膜带自身抗体研究近况

研究发现,瘢痕性类天疱疮、大疱性类天疱疮、获得性大疱表皮松解症、大疱性系统性红斑狼疮及线状IgA大疱病等表皮下大疱病均存在IgA型基底膜带自身抗体。线状IgA大疱病与瘢痕性类天疱疮、大疱性类天疱疮、获得性大疱表皮松解症不仅IgA型基底膜带自身抗体在基底膜带处沉积和结合方式相似,而且IgA型基底膜带自身抗体的靶抗原亦可相互重叠。大疱性类天疱疮及大疱性系统性红斑狼疮IgA型基底膜带自身抗体与疾病严重程度相关。     

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     

IgA型基底膜带自身抗体研究近况

研究发现，瘢痕性类天疱疮，大疱性类天疱疮，获得性大疱表皮松解症，大疱性系统性红斑狼疮及线状ＩｇＡ大疱病等表皮下大疱病均存在ＩｇＡ型基底膜带自身抗体。线状ＩｇＡ大疱病与瘢痕性类天疱疮，大疱性类天疱疮，获得性大疱表皮松解症不仅ＩｇＡ型基底膜带自身抗体在基底膜带处沉积和结合方式相似，而且ＩｇＡ型基底膜带自身抗体的靶抗原亦可相互重叠。     

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。     

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.     

用石蜡切片行C3d、C4d免疫组化染色辅助诊断大疱性类天疱疮的意义

目的 探讨C3d、C4d免疫组化染色在石蜡包埋组织切片中辅助诊断大疱性类天疱疮的价值.方法 通过免疫组织化学SP法检测20例大疱性类天疱疮患者石蜡包埋组织切片中C3d、C4d的表达,并与家族性良性天疱疮、大疱性表皮松解症患者及正常皮肤进行对照.结果 20例大疱性类天疱疮患者石蜡包埋切片C3d、C4d真表皮交界基底膜处沉积率为95％(19/20),9例大疱性表皮松解症患者中真表皮交界基底膜处C3d、C4d阳性率0％(0/9),4例家族性慢性良性天疱疮患者基底膜带均为阴性.结论 石蜡包埋组织切片中,C3d、C4d免疫组化染色可以作为辅助诊断大疱性类天疱疮的方法之一.     

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.     

Increased gliadin antibodies in dermatitis herpetiformis and pemphigoid

The mean titres of serum IgG and IgA gliadin antibodies were significantly increased in thirty-four patients with bullous pemphigoid, and in twenty-three patients with dermatitis herpetiformis, compared with twenty-four healthy controls. The patients with pemphigoid also had increased IgG and IgA gliadin antibodies compared with nine patients with pemphigus. The reason for the high titres of gliadin antibodies in pemphigoid is obscure. These patients may have increased intestinal permeability (suggested by the presence of beta-lactoglobulin antibodies in some patients). Alternatively gliadin may somehow precipitate the autoimmune process.     

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。     

Pitfalls in the diagnosis of pemphigus vulgaris (early pemphigus vulgaris with features of bullous pemphigoid)

This report draws attention to pitfalls that may be encountered in histologic diagnosis of early stages of pemphigus vulgaris. A man is described who had clinical and histologic features of bullous pemphigoid to begin with and later typical features of pemphigus vulgaris. Intercellular antibodies were demonstrable by direct immunofluorescence from the beginning.     

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.     

Pemphigus-like antibodies in bullous pemphigoid.

A patient had clinical, histopathologic, and direct immunofluorescent findings compatible with a diagnosis of bullous pemphigoid. In addition, the patient's serum contained pemphigus-like antibodies with different antigenic specificities than true pemphigus antibodies. Absorption of the patient's serum with AB substance failed to block the intercellular substance staining but allowed the demonstration of a basement membrane zone reactive antibody as well. Atypical cases such as this should not cast doubt on the usefulness of immunofluorescence in the diagnosis of bullous skin diseases.     

Case of bullous pemphigoid coexisting with anti‐desmoglein autoantibodies

A 79‐year‐old Japanese woman had clinical and histopathological features of bullous pemphigoid, while direct immunofluorescence test revealed C3 and immunoglobulin G depositions in the lower cell surfaces of the epidermis in addition to those in the dermoepidermal junction. Chemiluminescent enzyme immunoassays were positive for desmoglein‐1 and ‐3 antibodies in addition to anti‐BP180 antibodies. In an immunoblotting study, antibodies against both 180‐kDa bullous pemphigoid antigen and 130‐kDa pemphigus vulgaris antigen were detected. Based on these results, bullous pemphigoid coexisting with anti‐desmoglein autoantibodies was diagnosed in this case.     

Bullous pemphigoid and penicillamine

We describe a woman who developed bullous pemphigoid in association with penicillamine therapy. While pemphigus is a well-recognized complication of penicillamine, pemphigoid should also be considered in the differential diagnosis of bullous eruptions induced by this drug.     

Autoantibodies to bullous pemphigoid antigen 180 induce dermal-epidermal separation in cryosections of human skin

Bullous pemphigoid is a subepidermal autoimmune blistering disease associated with autoantibodies to the hemidesmosomal bullous pemphigoid antigens 180 and 230. Most sera from bullous pemphigoid patients recognize epitopes within the N-terminal NC16A portion of the bullous pemphigoid 180 ectodomain. Using cryosections of human skin, patients' sera were shown to generate dermal-epidermal separation when coincubated with leukocytes and complement from healthy volunteers; however, the specificity of pathogenic autoantibodies in bullous pemphigoid patients has not yet been elucidated. In this study, by the use of a modified version of the cryosection model, we show that sera from all of 13 bullous pemphigoid patients and from two rabbits, immunized against bullous pemphigoid 180 NC16A, induced dermal-epidermal separation. This finding was confirmed with the use of IgG purified from patients' sera, whereas sera and purified IgG from healthy controls were not pathogenic. The induction of subepidermal splits in this experimental model was shown to be dependent on the presence of neutrophils, but not complement. Interestingly, patients' autoantibodies affinity purified against a recombinant form of bullous pemphigoid 180 NC16A retained their blister-inducing capacity, whereas patients' IgG depleted of reactivity to NC16A lost this ability. F(ab')2 fragments of antibodies specific to NC16A, lacking the Fc portion, did not induce splits. In addition, patients' autoantibodies purified against a recombinant fragment of the C-terminus of bullous pemphigoid 180 as well as rabbit antibodies to the intracellular portion of bullous pemphigoid 180 and to bullous pemphigoid 230 did not cause dermal-epidermal separation. Our in vitro results support the idea that autoantibodies to bullous pemphigoid 180 from patients with bullous pemphigoid are of pathogenic relevance.