Omalizumab as an alternative therapeutic tool in the treatment of bullous pemphigoid: A case report

Bullous pemphigoid (BP) is an acquired autoimmune bullous disease characterized by autoantibodies against the hemidesmosomal proteins found in the basal keratinocytes of the basement membrane zone (BMZ): a 180 kDa protein (type XVII collagen) mainly and the 230 kDa antigen. There is such evidence that the antibodies against the BMZ components are not only of IgG type, but also this bullous disease may have IgE antibodies directed to the BMZ that contribute to the pathogenesis of the disorder. IgE is not only thought to contribute to the pathogenesis of BP, it has also been suggested that eosinophils play a role in the development of the first signs associated with BP. A humanized monoclonal antibody directed to IgE, omalizumab, is approved for the treatment of severe asthma and chronic spontaneous urticaria, and it may be useful in the treatment of BP in the first stages of the disease.     

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。     

大疱性类天疱疮与HLA—A、B等位基因的相关性

目的：确定山东汉族大疱性类天疱疮（BP）与HLA—A、B等位基因的相关性。方法：运用聚合酶链反应-序列特异性引物寡核苷酸探针杂交（PCR—SSOP）方法,对山东地区43例汉族BP患者和125例健康对照进行了HLL—A、B等位基因分型。结果：BP患者组HIA—A＊24频率高于对照组（P：0．033,Pc〉0．05）;HLA—A＊33、B＊44在患者组中频率低于正常对照组（P值分别为0．040和0．024,但Pc值均〉0．05）。结论：大疱性类天疱疮的遗传易感基因可能与HLA—A、B等位基因无相关性。     

Serum and blister fluid levels of cytokines and chemokines in pemphigus and bullous pemphigoid

Bullous pemphigoid and pemphigus constitute two major autoimmune blistering diseases (AIBD) with complicated disease pathomechanisms involving a multitude of cytokines and immunological pathways. The purpose of our literature review of the cytokines and chemokines involved in these AIBDs was to allow for a meta-analysis of studies detailing differential cytokine and chemokine changes in these conditions. Elucidation of inflammatory pathways could lead to more targeted therapies, several of which specific monoclonal antibodies already exist and are used safely for other autoimmune diseases. A systematic review of the Pubmed/Medline database was performed for articles characterizing cytokines/chemokines involved in BP and pemphigus. Further, a meta-analysis was carried out using standardized methods, including assessment for heterogeneity. The results of our analysis demonstrated numerous inflammatory alterations in these AIBDs. Significant alterations included serum levels of IL-5, IL-6, IL-8, IL-17, CCL-17, and CCL-26 in patients with BP, and increased blister fluids levels of IL-5, IL-6, IL-8, CCL11, and TNF-α. Blister fluid levels of IL-1α are decreased in BP. In pemphigus, we identified significantly increased serum levels of IL-10, IL-17, and CCL17. We have additionally summarized all studies excluded from meta-analysis to provide a comprehensive summary of cytokine/chemokine alterations in these two conditions.     

Juvenile bullous pemphigoid: the presentation and follow‐up of six cases

Background Juvenile bullous pemphigoid (JBP) is a very rare autoimmune blistering disease. Up to now, 81 cases have been published. Objectives To describe clinical, histopathological and immunopathological characteristics of our patients with JBP, their response to therapy and clinical course, and to show the rarity of JBP in our population. Study design Retrospective study of all patients with JBP diagnosed from 1983 to the end of 2007. The patients were followed from 6 months to 24 years. Setting An academic, teaching hospital – Pediatric Dermatology Unit of the Institute of Dermatovenereology, Clinical Center of Serbia, Belgrade. Patients and methods Six patients with JBP (4 girls and 2 boys) aged 2 to 17 years. The average age at onset of BP was 10 years. The diagnosis was confirmed by histopathological analysis and direct immunofluorescence test. The patients were treated with systemic and topical corticosteroids, and three patients received dapsone. Results The disease control was achieved after 2 weeks to 2.5 months of therapy. The treatment lasted from 2 weeks to 4.5 months, 1.8 months at the average. The period from the beginning of the disease to complete remission ranged from 1.5 month to 5 years, with a mean value of 14 months. In one boy, the parents interrupted the therapy, and the disease remitted spontaneously after 5 years. Conclusion According to our experience, JBP can spontaneously remit within 5 years. JBP has a relatively indolent course and may be a self‐limiting disease. The patients should not be over‐treated in order to avoid side‐effects of medication.     

Dipeptidyl peptidase‐4 inhibitor‐associated bullous pemphigoid in a patient with acquired reactive perforating collagenosis

Bullous pemphigoid (BP) is a common autoimmune blistering disorder with unknown etiology. Recently, increasing numbers of BP cases which developed under the medication with dipeptidyl peptidase‐4 inhibitors (DPP4i), widely used antihyperglycemic drugs, have been reported in published works. Here, we report a case of DPP4i (teneligliptin)‐associated BP that developed in a 70‐year‐old Japanese man. Interestingly, the patient had acquired reactive perforating collagenosis (ARPC), which is also known to be associated with the onset of BP. In the present case, clinical, histopathological and immunological findings suggested that DPP4i rather than ARPC was associated with the onset of BP.