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三级医疗中心各类自身免疫性水疱性疾病的临床流行病学研究

A Clinico-Epidemiological Study of Various Autoimmune Vesiculobullous Disorders in a Tertiary Care Center.

作者信息

Sri Koulini Vunnava, Chintada Dilipchandra, Vudayana Kirankanth, Bathina Aruna

机构信息

Department of Dermatology, Venereology and Leprosy, Great Eastern Medical School and Hospital, Srikakulam, IND.

出版信息

Cureus. 2025 Jun 22;17(6):e86571. doi: 10.7759/cureus.86571. eCollection 2025 Jun.

DOI:10.7759/cureus.86571
PMID:40709122
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12286763/
Abstract

Background Pemphigus and pemphigoid disorders, as well as dermatitis herpetiformis, are all part of the diverse group of conditions known as autoimmune vesiculobullous/blistering disorders (AIBDs). These disorders are caused by autoantibodies that target intercellular or cell-matrix adhesion proteins and are clinically characterized by blisters or erosions of the skin and/or mucous membranes. The incidence peaks at 30-60 years with an equal incidence in men and women. There are 0.1 to 0.5 cases of pemphigus for every 100,000 people. Materials and methods This single-center retrospective observational study was conducted at our tertiary care center (Great Eastern Medical School and Hospital) in Srikakulam, Andhra Pradesh, India, between March 2018 and March 2025, and examined 50 histopathologically and direct immunofluorescence confirmed cases of autoimmune vesiculobullous disorders over a seven-year period. Results The percentage of females, i.e., 28 (56%) out of 50 individuals, is marginally higher than that of males (22 (44%)). The most prevalent age group is 51-60 years old (26%), followed by 41-50 years old (20%). The majority of cases are from rural areas (72%) and are occupied by people from low socioeconomic backgrounds (72%). The majority of farmers are local residents (76%) with few migrants (24%). Of the 50 cases, the most prevalent intraepidermal variants were pemphigus vulgaris (18 cases) and pemphigus foliaceus (nine cases), with a single case of pemphigus vegetans, whereas the most prevalent sub-epidermal variants were bullous pemphigoid (14 cases), with a small number of epidermolysis bullosa acquisita cases (eight cases). In 44% of instances, mucosal involvement is observed. Comorbidities, such as diabetes, hypertension, heart disease, and malignancies, were found in a small percentage of cases. Corticosteroids were the first line of treatment, and immunosuppressants were used for maintenance. Few patients had positive outcomes using dexamethasone cyclophosphamide pulse therapy (DCP), and rituximab given in two cases showed disease control after the second dose. Better disease control was demonstrated by patients who adhered to the long-term treatment. Conclusion In recent years, autoimmune vesiculobullous illnesses have become more prevalent and are significantly correlated with other systemic conditions. A difficult problem that requires careful handling is atypical clinical manifestations, which can be linked to inappropriate and delayed treatment as well as noncompliance. Long-term steroid medication is less necessary with early diagnosis, which further minimizes consequences by evaluating the disease activity and severity and starting a targeted treatment. For the general improvement of quality of life, public education, development of medical services in rural regions, and the elimination of social stigma are, therefore, crucial.

摘要

背景 天疱疮和类天疱疮疾病,以及疱疹样皮炎,均属于自身免疫性水疱性/大疱性疾病(AIBDs)这一多样的疾病组。这些疾病由针对细胞间或细胞基质黏附蛋白的自身抗体引起,临床特征为皮肤和/或黏膜出现水疱或糜烂。发病率在30 - 60岁达到峰值,男女发病率相等。每10万人中有0.1至0.5例天疱疮病例。

材料与方法 本单中心回顾性观察研究于2018年3月至2025年3月在印度安得拉邦斯里卡库拉姆的我们的三级医疗中心(大东方医学院和医院)进行,在七年期间检查了50例经组织病理学和直接免疫荧光确诊的自身免疫性水疱性疾病病例。

结果 女性比例,即50人中的28人(56%),略高于男性(22人(44%))。最常见的年龄组是51 - 60岁(26%),其次是41 - 50岁(20%)。大多数病例来自农村地区(72%),且患者多来自社会经济背景较低的群体(72%)。大多数农民是当地居民(76%),移民较少(24%)。在50例病例中,最常见的表皮内型是寻常型天疱疮(18例)和落叶型天疱疮(9例),有1例增殖型天疱疮,而最常见的表皮下型是大疱性类天疱疮(14例),获得性大疱性表皮松解症病例较少(8例)。在44%的病例中观察到黏膜受累。在一小部分病例中发现有合并症,如糖尿病、高血压、心脏病和恶性肿瘤。皮质类固醇是一线治疗药物,免疫抑制剂用于维持治疗。少数患者使用地塞米松环磷酰胺脉冲疗法(DCP)有阳性结果,2例使用利妥昔单抗的患者在第二剂后病情得到控制。坚持长期治疗的患者病情控制更好。

结论 近年来,自身免疫性水疱性疾病变得更加普遍,并且与其他全身性疾病显著相关。非典型临床表现是一个需要谨慎处理的难题,这可能与不适当和延迟的治疗以及不依从性有关。早期诊断减少了长期使用类固醇药物的必要性,通过评估疾病活动度和严重程度并开始针对性治疗进一步将后果最小化。因此,为了总体提高生活质量,公众教育、农村地区医疗服务的发展以及消除社会耻辱感至关重要。

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本文引用的文献

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Rituximab in the Treatment of Epidermolysis Bullosa Acquisita: A Systematic Review of the Literature.利妥昔单抗治疗获得性大疱性表皮松解症:文献系统评价
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