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首页> 外文期刊>Clinics in dermatology >Pemphigus: Associations and management guidelines: Facts and controversies
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Pemphigus: Associations and management guidelines: Facts and controversies

机译：天疱疮：协会和管理准则：事实与争议

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Pemphigus, a prototypical organ-specific human autoimmune disease, may be associated with other immunity-related disorders, viral infections, and different types of tumors. Coexistence with immune diseases is fairly frequent and, for some of them (eg, myasthenia gravis, Basedow's disease, rheumatoid arthritis, or lupus erythematosus), common pathogenic mechanisms can be considered. The association with viral infections (mainly herpesvirus infections) raises the question of whether the virus triggers the outbreak of the disease or simply complicates its clinical course. Neoplastic proliferations coexisting with pemphigus have a different histogenesis and the pathogenic link may vary according to the associated tumor (thymoma, lymphoma, carcinoma, or sarcoma). A subset of pemphigus-neoplasia association is represented by Anhalt's paraneoplastic pemphigus, with peculiar clinical, histologic, and immunologic features characterizing it. Coexistence of pemphigus with Kaposi's sarcoma, albeit not frequent, offers an intriguing speculative interest. The cornerstone of management in pemphigus is the combination of systemic corticosteroids and immunosuppressants. The conventional treatment used in most cases is based on oral administration of deflazacort and azathioprine. In selected cases, mycophenolate mofetil is preferred to azathioprine. Severe forms of pemphigus require intravenous pulse therapy with dexamethasone (or methylprednisolone) and cyclophosphamide.In the recent years, the use of high-dose intravenous immunoglobulin therapy has gained several consents. Rituximab, a monoclonal anti-CD 20 antibody, which affects both the humoral and cell-mediated responses, has proved to give a good clinical response, often paralleled by decrease of pathogenic autoantibodies. The combination with intravenous immunoglobulin offers the double advantage of better clinical results and a reduced incidence of infection.Interventional treatments, such as plasmapheresis and extracorporeal immunoadsorption, are aimed at patients with life-threatening forms of pemphigus and high levels of circulating autoantibodies, a circumstance where the medical therapy alone risks failing. Second-line treatments include gold salts (which we do not favor because of the acantholytic potential inherent in thiol structure) and the association of oral tetracyclines with nicotinamide, which is rather safe.Local treatments, supplementary to the systemic therapy, are aimed at preventing infections and stimulating reepithelialization of eroded areas. Innovative topical treatments are epidermal growth factor, nicotinamide gel, pimecrolimus, and a proteomics-derived desmoglein peptide.Pemphigus patients should be warned against over-indulging in unnecessary drug intake, prolonged exposure to ultraviolet rays, intense emotional stress, and too spiced or too hot foods. Cigarette smoking is not contraindicated in pemphigus patients because of the nicotine anti-acantholytic properties.
机译：天疱疮，一种典型的器官特异性人类自身免疫性疾病，可能与其他与免疫相关的疾病，病毒感染和不同类型的肿瘤有关。与免疫性疾病共存的情况相当普遍，对于其中一些疾病（例如重症肌无力，巴氏病，类风湿性关节炎或红斑狼疮），可以考虑常见的致病机制。与病毒感染（主要是疱疹病毒感染）的关系引发了一个问题，即病毒是引发疾病的爆发还是使其临床过程变得简单。与天疱疮共存的赘生性增生具有不同的组织发生，其致病作用可能因相关肿瘤（胸腺瘤，淋巴瘤，癌瘤或肉瘤）而异。天疱疮-neoplasia协会的一个子集是由Anhalt的副肿瘤性天疱疮所代表，具有特有的临床，组织学和免疫学特征。天疱疮与卡波西氏肉瘤并存，尽管并不常见，却引起了人们的兴趣。天疱疮的治疗基础是全身性皮质类固醇和免疫抑制剂的结合。在大多数情况下使用的常规治疗是基于口服黄酮和硫唑嘌呤。在某些情况下，霉酚酸酯优于硫唑嘌呤。严重的天疱疮需要使用地塞米松（或甲基强的松龙）和环磷酰胺进行静脉内脉冲治疗。近年来，大剂量静脉内免疫球蛋白治疗已获得了一些同意。利妥昔单抗是一种单克隆抗CD 20抗体，可影响体液和细胞介导的反应，已被证明具有良好的临床反应，通常与致病性自身抗体减少相伴。静脉免疫球蛋白的结合具有更好的临床效果和降低的感染率的双重优势。血浆置换术和体外免疫吸附等介入治疗的对象是危及生命的天疱疮和高水平循环自身抗体的患者。仅药物治疗就有失败的风险。二线治疗包括金盐（由于硫醇结构固有的棘阿德酸潜力，我们不赞成使用）和口服四环素与烟酰胺的联合使用，这是相当安全的。作为全身治疗的补充的局部治疗旨在预防感染和刺激侵蚀区域的再上皮化。创新的局部治疗方法是表皮生长因子，烟酰胺凝胶，吡美莫司和蛋白质组学衍生的桥粒糖蛋白肽。应警告天疱疮患者不要过度沉迷不必要的药物摄入，长时间暴露于紫外线，强烈的情绪压力以及太辣或太辣。热食。天疱疮患者不禁止吸烟，因为尼古丁具有抗棘皮分解作用。

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