■自身免疫性大疱性疾病(AIBDs)是一组罕见的皮肤疾病,影响角化的皮肤和粘膜。它们的特征是由于针对皮肤的桥粒和半桥粒结构蛋白的自身抗体引起的紧张或松弛的起泡和侵蚀。这组疾病可分为类天疱疮和天疱疮疾病。如果不及时治疗,这些自身免疫性疾病会导致严重甚至危及生命的并发症,如液体流失,超感染或食物摄入受损。由于现代标准化的血清学检测,AIBDs的诊断通常可以结合其临床表现来确认。而长期以来,皮质类固醇是治疗这些疾病的主要参与者,在利妥昔单抗和其他免疫抑制剂的批准下,治疗日益改善。
■在这项研究中,我们的目的是调查中Franconia大疱性自身免疫性疾病的流行病学和临床特征以及诊断和治疗,德国联邦巴伐利亚州的一个省。这项回顾性研究包括在大学医院Erlangen皮肤科因01.04.2013至31.03.2019之间的AIBDs而诊断或治疗的患者(n=242)。患者要么首次被诊断(n=176),要么已在该部门确认诊断(n=66)。在此期间,在该中心被诊断出的176名受试者中计算了各自的发病率。数据取自患者记录并用Microsoft®Excel分析。评估包括寻常型天疱疮(PV)的诊断,天疱疮(PF),大疱性类天疱疮(BP),粘膜类天疱疮(MMP),线性IgA皮肤病(LAD),大疱性表皮松解症(EBA),和疱疹样皮炎(DH)。
■这项研究表明,中弗兰肯州每个AIBDs的发生率都很低且具有可比性(PV,PF,LAD,EBA)或更低(BP,MMP,DH)比其他研究和地区。BP是中Franconia最常见的新诊断的AIBD。
■由于AIBDs的慢性且有时严重的病程,重复的内部治疗通常是必要的。迄今为止,主要局部和全身应用皮质类固醇与免疫调节剂联合作为一线治疗。
Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved.
In this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH).
This study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia.
Due to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.