天疱疮合并症的研究进展

天疱疮是一种以表皮内棘层松解为特征的自身免疫性大疱性皮肤病。天疱疮可以合并银屑病、神经系统疾病、血液系统疾病、其他自身免疫性疾病以及肿瘤等。近年来有关天疱疮合并症的报道越来越多,本文对天疱疮合并症的研究进展作一综述。     

糖皮质激素对天疱疮和类天疱疮患者诱发糖尿病的临床研究北大核心CSCD

目的探讨糖皮质激素治疗后诱发糖尿病的天疱疮患者和类天疱疮患者的临床特征及其糖皮质激素受体水平变化情况。方法根据是否出现糖尿病,将接受糖皮质激素治疗的22例天疱疮患者和17例类天疱疮患者分为两组(糖尿病组和非糖尿病组),比较两组患者的一般资料、皮损愈合情况和糖皮质激素受体检测情况。结果糖尿病组患者体质指数(25.02±1.97 vs 21.77±1.35)、激素起始剂量(59.21±1.89 vs 40.45±2.11)、每日激素最大剂量(100.23±2.23 vs 61.92±2.03)、激素治疗时间(14.22±1.76 vs 2.45±0.34)均高于非糖尿病组;止疱时间(85±3.11 vs 115±2.89)、结痂时间(180±9.66 vs 220±5.12)、脱痂时间(210±4.22 vs 250±5.02)均低于非糖尿病组。治疗后,糖尿病组患者皮损中GRα表达量显著增加,非糖尿病患者皮损中GRα的表达量无明显变化,与治疗前比较,差异均有统计学意义(P=0.002)。结论糖皮质激素治疗天疱疮和类天疱疮能够提高患者的体质指数,缩短患者的止疱、结痂和脱痂时间,为临床使用糖皮质激素治疗提供参考依据。     

血浆置换疗法治疗天疱疮、大疱性类天疱疮疗效分析

为探讨血浆置换疗法治疗天疱疮及大疱性类天疱疮的疗效；回顾性分析曾接受血浆置换疗法的10例天疱疮、7例大疱性类天疱疮患者的临床资料。结果：10例天疱疮患者血浆置换后皮损均不同程度减轻，其中4例尼氏征数日内由阳性迅速转为阴性；5例全身症状好转；4例检测天疱疮抗体滴度者，3例下降，1例无改变；3例糖皮质激素用量减少，另1例第三次血浆置换后糖皮质激素用量比第一次减少。7例大疱性类天疱疮患者血浆置换后皮损均减轻；4例全身症状减轻；4例糖皮质激素用量减少；血浆置换疗法辅助治疗天疱疮及大疱性类天疱疮可取得较好的疗效。     

副肿瘤性类天疱疮的研究进展

大疱性类天疱疮与恶性肿瘤之间存在相关性,其相关恶性肿瘤类型包括血液系统肿瘤、前列腺癌、胃癌等,大部分恶性肿瘤出现在大疱性类天疱疮皮损之时或之后.副肿瘤性类天疱疮发病机制尚不清楚,治疗与经典大疱性类天疱疮有一定区别,患者皮损随着肿瘤的控制与治愈而好转.因此,临床中应注意大疱性类天疱疮患者可能伴发的恶性肿瘤,做到早发现早诊断早治疗,提高患者生命质量.     

环孢素治疗银屑病并发天疱疮一例

银屑病并发天疱疮临床少见,两种疾病治疗存在矛盾。该例患者有银屑病病史7年,20 d前出现水疱、糜烂;皮损组织病理及疱病自身抗体检查支持寻常性银屑病并发寻常性天疱疮诊断,给予口服环孢素及外用药物治疗后病情改善。     

大疱性类天疱疮1例

大疱性类天疱疮是一种获得性自身免疫性大疱性皮肤病,好发于老年人,多表现为累及身体屈侧的张力性水疱、大疱,伴不同程度的瘙痒.发病的早期和恢复期,大疱性类天疱疮的皮损常可呈多形性,临床易误诊.现报告1例大疱性类天疱疮误诊为多形性日光疹.     

10例寻常型银屑病合并大疱性类天疱疮临床分析

目的：总结寻常型银屑病合并大疱性类天疱疮患者的临床特征及治疗方法。方法：回顾性分析2016年1月至2018年12月我院住院患者中诊断为寻常型银屑病合并大疱性类天疱疮的临床资料。结果：共发现10例寻常型银屑病合并大疱性类天疱疮患者,其中男9例,女1例,平均年龄为（59.90±10.18）岁,银屑病均先于大疱性类天疱疮发病。8例患者水疱发生于银屑病斑块上,2例水疱发生于外观正常的皮肤上。9例患者采用糖皮质激素和/或免疫抑制剂治疗,1例患者应用口服四环素、烟酰胺配合光疗治疗。出院后随访150~1065天,7例患者无新发水疱,银屑病皮损病情稳定;2例失访,1例死亡。结论：寻常型银屑病合并大疱性类天疱疮临床相对少见,中老年患者居多,糖皮质激素联合免疫抑制剂治疗有效。     

得宝松皮损内注射治疗天疱疮和大疱性类天疱疮口腔粘膜损害

使用皮损内注射得宝松 (复方倍他米松 )治疗天疱疮、大疱性类天疱疮，并与未使用此疗法治疗的患者作一比较。口腔粘膜损害使用局部注射得宝松治疗组 (天疱疮＋大疱性类天疱疮 )29例，口腔粘膜损害平均愈合时间为 0.8± 0.8月；口腔粘膜损害未用局部注射得宝松治疗组 (天疱疮＋大疱性类天疱疮 )31例，口腔粘膜损害平均愈合时间为 1.5± 1.3月。经统计学比较分析 t=2.49， P< 0.01，两组口腔粘膜损害平均愈合时间具有显著性差异，前组明显短于后组。采用皮损内注射得宝松治疗口腔粘膜的顽固损害，药物直达病灶，疗效快，并可减少系统用药量，减少药物副作用。     

天疱疮282例和类天疱疮366例回顾性研究

目的 探讨天疱疮和类天疱疮诊断与治疗特点.方法 对2003-2007年天疱疮和类天疱疮648例患者的临床资料进行同顾性分析.结果 天疱疮平均发病年龄低于类天疱疮(P＜0.001),直接免疫荧光(DIF)确诊的175例天疱疮患者中IgG100%阳性,C3 92.0%阳性;类天疱疮223例患者中C399.1%阳性,IgG 51.12%阳性.两种大疱性疾病的诊断中,①天疱疮患者的临床诊断、病理检查与DIF三者一致率为68.8%.病理检查、DIF两者一致率为80.7%.②类天疱疮患者的临床诊断、病珲检查与DIF三者一致率为62.8%.病理检查、DIF两者一致率为78.1%.糖皮质激素是治疗两种疾病的主要手段,泼尼松(0.5～1.5)mg·kg-1·d-1可控制大部分患者病情.结论 临床和病理无法确诊的大疱疮和类天疱疮病例,DIF可以作为诊断该病的重要方法.在基层医院,临床怀疑天疱疮,DIF可主要检查IgG、C3,怀疑类天疱疮,可主要检查C3.     

寻常性银屑病合并结节性类天疱疮1例并文献复习

报告1例寻常性银屑病合并结节性类天疱疮。患者男，56岁，既往有银屑病病史8年。全身红斑、风团反复发作1年余，双上肢丘疹伴瘙痒1个月。皮肤科检查：头皮散在红斑，上覆鳞屑；背部及四肢散在红色斑块，边界清楚，上覆银白色鳞屑；躯干及四肢大片红斑、风团；头部、背部及四肢暗褐色丘疹、结节，未见水疱、大疱及脓疱。皮损组织病理检查：角化过度，角化不全，表皮不规则增生，表皮下可见裂隙，真皮浅层血管周围可见淋巴细胞及少量嗜酸性粒细胞浸润。间接免疫荧光：IgG沉积于真表皮交界处。酶联免疫吸附试验（ELISA）：抗大疱性类天疱疮抗原1(BP230)抗体和抗大疱性类天疱疮抗原2(BP180)抗体阳性。诊断：寻常性银屑病合并结节性类天疱疮。     

自身免疫性疱病与银屑病的相关性

自身免疫性疱病（AIBD）和银屑病的发病机制有部分相似性。近年来,多项研究报道银屑病与AIBD之间具有相关性,以大疱性类天疱疮多见,还包括寻常型天疱疮、红斑型天疱疮、线状IgA大疱性皮病等,多数AIBD在银屑病发病后发生,部分患者两病同时出现或AIBD先出现。本文综述AIBD与银屑病发病的相关性及可能存在的机制。     

The International Pemphigus and Pemphigoid Foundation

The International Pemphigus Pemphigoid Foundation (IPFF) was founded in 1997. The IPPF lists more than 4500 members. The IPPF provides peer health coaches to aid patients in the navigation of the health care system and recommends dermatologists and other specialists in their area who are experts in autoimmune bullous disease. The IPPF hosts the largest worldwide registry of pemphigus/pemphigoid patients with biospecimen collection opportunities are planned. Twice a year the IPPF hosts formal meetings with invited speakers.     

Complement in pemphigus vulgaris and other bullous dermatoses

Summary The behaviour of the complement and of its components C4 and C3 has been studied in serum and in blister fluid both in patients with pemphigus vulgaris and other bullous dermatoses and in healthy subjects with experimentally induced blisters.The results are suggestive of local activation of the complement, in the blister fluid of patients with pemphigus vulgaris, pemphigus erythematosus and bullous pemphigoid according to the classical enzymatic sequence.

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Zusammenfassung Die Aktivität von Komplement, insbesondere der C3-und C4-Komponenten wurde im Serum und im Blaseninhalt bei Patienten mit Pemphigus vulgaris und anderen bullösen Dermatosen sowie bei gesunden Kontrollpersonen mit experimentell ausgelösten Blasen untersucht.Die Ergebnisse sprechen für eine lokale Aktivierung von Komplement im Sinne der klassischen Enzymsequenz bei Patienten mit Pemphigus vulgaris, Pemphigus erythematosus und beim bullösen Pemphigoid.

     

Quality of Life Assessment in Korean Patients with Pemphigus

Background

Measuring the quality of life (QOL) is important in the evaluation of nonclinical aspects of diseases, for the discovery of functional and psychological limitations, and in choosing treatment in the initial phase of the disease. Pemphigus is a potentially fatal autoimmune bullous disease caused by autoantibodies against desmogleins (cadherin family proteins in desmosomes). Thus far, there has been no published study on QOL in Korean patients with pemphigus.

Objective

To study the impact of pemphigus on the QOL in a large number of Korean patients.

Methods

Sixty-six patients enrolled at the Gangnam Severance Hospital from March 2012 to March 2013 were assessed for QOL by using the Dermatology Life Quality Index (DLQI), and for anxiety and depression by using the General Health Questionnaire (GHQ). Spearman''s rank-order correlation, t-test, and ANOVA were used to identify the relations between the DLQI score and other clinical variables.

Results

Pemphigus vulgaris and pemphigus foliaceus significantly reduced the QOL of patients. The average DLQI score for all patients was 10.18. The mean DLQI score was 13.45 in patients in the active state and 5.15 in the patients in the remission state. The DLQI score highly correlated with disease severity, titer of anti-desmoglein 1 in enzyme-linked immunosorbent assay, and the corticosteroid dose. However, the QOL was not affected by sex, age, subtype of pemphigus, duration of disease, or comorbidities. Forty-two percent of the patients showed a positive result in the GHQ, reflecting probable minor psychiatric nonpsychotic conditions, and the GHQ score positively correlated to the DLQI score.

Conclusion

Pemphigus significantly impairs the QOL of patients. The QOL of Korean pemphigus patients significantly correlates with clinical severity. Therefore, considerable attention should be paid to the patients'' QOL and psychological states as well as clinical status.     

Ontogeny of pemphigus and bullous pemphigoid antigens in human skin

The development of pemphigus and bullous pemphigoid antigens in twenty-nine human foetuses aged 9–40 weeks has been examined by immunofiuorescence. Pemphigus antigen was weakly demonstrable in the epidermal intercellular area at 9 weeks and strongly after 12 weeks, Bullous pemphigoid antigen was weak and patchy at 12 weeks and only consistently demonstrable with bright linear immunofiuorescence of the basement membrane zone after 23 weeks. Morphologically detectable epidermal basement membrane is already present by 9 weeks gestation.     

Deutliche Einschränkung der Lebensqualität bei Patienten mit Pemphigus vulgaris: Ergebnisse der deutschen Bullous Skin Disease (BSD)‐Studiengruppe

Background: Pemphigus vulgaris is a potentially life‐threatening autoimmune disorder of the skin and mucous membranes characterized by antibodies against epidermal adhesion molecules. Clinically characteristic are painful chronic blisters or erosions of mucous membranes and skin. There are no published studies on the impact o this disease on quality of life. Patients and methods: This registration was performed within the scope of the German BSD (Bullous Skin Disease) study group, from November 1997 until January 2002. A total of 36 patients with the first diagnosis of pemphigus vulgaris were registered at the university hospitals of Dresden, Erlangen, Kiel, Mannheim, München and Würzburg. Thirty of the 36 (83 %) patients participated in the quality of life questionnaire utilizing the German version of ‘Dermatology Life Quality Index’ (DLQI) provided by A. Y. Finlay. The DLQI varies from 0 to 30 with an increased DLQI score indicating a decrease in quality of quality. Results: The overall DLQI total score of 10 ± 6,7 in the investigated pemphigus patients was significantly increased in comparison to other skin diseases. Conclusions: These results suggest that the DLQI can be a very useful additional outcome criteria for clinical studies with pemphigus vulgaris and in the treatment of these patients.     

Neonatal Autoimmune Blistering Disease: A Systematic Review

We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.     

Autoimmune bullous skin diseases. Part 1: Clinical manifestations

Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders.     

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.