Autoimmune bullous diseases

自身免疫性大疱性疾病
  • 文章类型: Letter
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  • 文章类型: Journal Article
    背景与目的:利妥昔单抗(RTX)已成为自身免疫性大疱性疾病(AIBDs)的主要治疗药物。这项研究的目的是评估RTX治疗AIBD患者的优势和安全性特征。这项评估的重点是临床缓解和糖皮质激素使用的减少,其对靶向桥粒蛋白-1(DSG-1)和桥粒蛋白-3(DSG-3)的自身抗体滴度的影响,以及在中欧大学背景下的皮肤科进行的12个月随访期间的不良事件。材料与方法:我们的病例系列涉及11例患者,包括8名寻常型天疱疮患者,两个患有天疱疮,还有一个患有大疱性表皮松解症。他们接受了1克剂量的利妥昔单抗,在两周的间隔内重复。结果:第二次RTX输注后2、6和12个月泼尼松等效剂量的减少为65.05%,73.99%,76.93%,按这个顺序。抗DSG-1抗体的滴度降低了43.29%,75.86%,在2个月、6个月和12个月时为54.02%,分别。相比之下,靶向DSG-3的抗体浓度下降了27.88%,14.48%,和相应时间点的5.09%。在12个月的监测期间,18.18%的患者出现疾病复发,而其余个体在最小或不接受治疗的情况下实现了完全或部分缓解。36.36%的患者出现不良反应;它们是轻度的,未报告严重不良反应.结论:RTX是治疗AIBD的有效且耐受性良好的治疗选择,在难治性AIBD的情况下值得考虑。然而,进一步的研究是必要的,以划定最佳剂量,给药频率,以及所需的维持输液总量。此外,迫切需要进行研究,探索RTX对AIBD患者的影响,这些患者的抗桥粒蛋白自身抗体水平没有显著降低.
    Background and Objectives: Rituximab (RTX) has been the predominant treatment for autoimmune bullous diseases (AIBDs). The objective of this research was to assess the advantages and safety characteristics of RTX treatment in individuals with AIBD. This assessment focused on clinical remission and a reduction in glucocorticosteroid usage, its effect on the titers of autoantibodies targeting desmoglein-1 (DSG-1) and desmoglein-3 (DSG-3), and adverse occurrences during a 12-month follow-up period in a dermatology department within a Central European university context. Materials and Methods: Our case series involved eleven patients, including eight patients with pemphigus vulgaris, two with pemphigus foliaceus, and one with epidermolysis bullosa acquisita. They received a 1 g dose of rituximab, repeated over a two-week interval. Results: The reduction in a prednisone-equivalent dosage after 2, 6, and 12 months following the second RTX infusion was 65.05%, 73.99%, and 76.93%, in that order. The titers of antibodies against DSG-1 exhibited reductions of 43.29%, 75.86%, and 54.02% at 2, 6, and 12 months, respectively. By contrast, the antibody concentrations targeting DSG-3 displayed a decrease of 27.88%, 14.48%, and 5.09% at the corresponding time points. Over the course of the 12-month monitoring period, 18.18% of patients experienced disease relapse, while the remaining individuals achieved either complete or partial remission with minimal or no therapy. Adverse effects were noted in 36.36% of the patient population; they were mild, and no serious adverse effects were reported. Conclusions: RTX represents an efficacious and well-tolerated therapeutic option for the management of AIBD and merits consideration in cases of refractory AIBD. However, further research is imperative to delineate the most optimal dosage, dosing frequency, and total quantity of maintenance infusions required. Additionally, there is a compelling need for studies that explore the impact of RTX on individuals with AIBD who do not exhibit a significant reduction in anti-desmoglein autoantibody levels.
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  • 文章类型: Journal Article
    背景与目的:自身免疫性大疱性疾病(AIBDs)可以通过静脉免疫球蛋白(IVIG)输注来治疗。本研究旨在评估大剂量IVIG治疗AIBD患者的益处和安全性。根据临床缓解情况确定,保留糖皮质激素的作用,中欧大学皮肤科12个月随访时的不良事件。材料和方法:我们的病例系列包括10例患者:5例寻常型天疱疮,一个患有疱疹样天疱疮,一个有天疱疮,一个患有大疱性类天疱疮,两个患有大疱性表皮松解症。他们接受了4-12个月的IVIG治疗周期,每个周期的剂量为2g/kg。结果:最终IVIG疗程后2、6和12个月的泼尼松剂量减少为65.45%,70.91%,76.37%,分别。在12个月的观察期间,在20%的患者中观察到疾病复发,而其他人则在没有或最少治疗的情况下实现了完全或部分缓解。80%的患者出现副作用;它们是短暂的,不需要停用IVIG。结论:IVIG证明了作为一种具有良好安全性的治疗方法的有效性。然而,它的高成本仍然是一个显著的缺点,特别是在低收入国家。IVIG应该被考虑,特别是在反对标准疗法或有禁忌症的患者中。
    Background and Objectives: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-sparing effect, and adverse events at 12 months follow-up in a Central European university dermatology department setting. Materials and Methods: Our case series included 10 patients: five patients with pemphigus vulgaris, one with pemphigus herpetiformis, one with pemphigus foliaceus, one with bullous pemphigoid, two with epidermolysis bullosa acquisita. They underwent 4-12 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. Results: The prednisone dosage reduction after 2, 6, and 12 months following the final IVIG course was 65.45%, 70.91%, and 76.37%, respectively. During the 12-month observation period, disease relapse was observed in 20% of patients, while others achieved complete or partial remission without or with minimal therapy. Side effects were seen in 80% of patients; they were transient and did not necessitate discontinuation of IVIG. Conclusions: IVIG demonstrates effectiveness as a treatment with a favorable safety profile. Nevertheless, its high cost remains a significant drawback, particularly in low-income countries. IVIG should be considered, especially in patients opposed to standard therapies or with contraindications to their use.
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  • 文章类型: Case Reports
    银屑病是一种免疫介导的慢性炎症性疾病,可合并糖尿病等并发症,心血管疾病,肥胖,和肾脏疾病。以前已经报道过银屑病与自身免疫性大疱性疾病(AIBD)的共病,其中最常见的是大疱性类天疱疮(BP)。银屑病合并BP的潜在机制尚不清楚,也没有统一的治疗标准。根据以前的病例报告,银屑病与BP共存可能与炎症活动有关,药物,光疗,和感染。我们报告了一例银屑病患者,服用中草药化合物后出现BP,并成功使用dupilumab治疗,这是首例报道的应用dupilumab治疗伴有BP合并症的银屑病病例.
    Psoriasis is an immune-mediated chronic inflammatory disease that can be combined with complications such as diabetes, cardiovascular disease, obesity, and kidney disease. The comorbidity of psoriasis with autoimmune bullous diseases (AIBD) has been reported previously in several cases, the most frequent of which is bullous pemphigoid (BP). The underlying mechanisms of psoriasis with BP are not clear and there are no uniform treatment criteria. Based on previous case reports, the coexistence of psoriasis and BP may be related to inflammatory activity, medications, phototherapy, and infection. We report a case of a psoriasis patient who developed BP after taking Chinese herbal compounds and was successfully treated with dupilumab, which is the first reported case of applying dupilumab to treat psoriasis with BP comorbidities.
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  • 文章类型: Case Reports
    Bullous systemic lupus erythematosus (BSLE) is a rare blistering skin manifestation of systemic lupus erythematosus (SLE). Dapsone is reported to be helpful in mild-to-moderate BSLE cases; however, its use may be limited or prohibited due to particular complications such as drug hypersensitivity, dose-dependent hemolytic anemia, and other significant hematologic abnormalities. Rituximab, an anti-CD20 monoclonal antibody, has been reported with off-label use in BSLE patients, but data are still limited. Hence, our objective is to explore the efficacy of rituximab among these patients. Herein, we report a 21-year-old Thai woman presented with blistering eruption on the oral cavity, scalp, trunk, and extremities for 1 month. The investigations revealed a positive direct Coomb\'s test, an elevated erythrocyte sedimentation rate (ESR), and a positive antinuclear antibody (ANA). Skin biopsy showed focal interface dermatitis. Direct immunofluorescence (DIF) illustrated mixed linear and granular deposition of immunoglobulin (Ig)G, IgM, IgA, and C3 along the dermo-epidermal junction (DEJ). Enzyme-linked immunosorbent assay (ELISA) showed circulating antibodies to type VII collagen. She was diagnosed with severe BSLE and autoimmune hemolytic anemia (AIHA) refractory to several oral immunosuppressants but was successfully treated with rituximab. The authors also performed a review of the literature on prior BSLE cases managed with rituximab.
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  • 文章类型: Case Reports
    9个月前,一名15岁的日本男性在他的背上发现了褐色的斑斑。初步检查显示网状浸润性红斑和色素沉着,背部红斑上有水泡。组织病理学显示表皮有嗜酸性粒细胞浸润的水疱,直接免疫荧光显示免疫球蛋白(Ig)G阴性结果,IgA,IgM,和C3在表皮基底膜区。免疫血清学测试显示存在针对BP180的IgG抗体,线性IgA疾病抗原1(LAD-1),层粘连蛋白α3.怀疑是自身免疫性大疱性疾病,开始使用浓度为20mg/天(0.3mg/kg/天)的泼尼松龙。当泼尼松龙剂量减少到10毫克/天时,红斑和水疱复发。根据临床特征,该患者被诊断为色素性瘙痒,并口服盐酸多西环素水合物和外用他克莫司软膏成功治疗。这是第一例色素性瘙痒伴水疱,其中发现了表皮基底膜区的自身抗体,可能是二级非致病性抗体。
    A 15-year-old Japanese male noticed brown macules on his back 9 months ago. Initial examination revealed reticulated infiltrative erythema and pigmentation with blisters on the erythema of the back. Histopathology showed blisters with eosinophil infiltration in the epidermis, and direct immunofluorescence showed negative results for immunoglobulin (Ig) G, Ig A, Ig M, and C3 in the epidermal basement membrane zone. Immuno-serological tests revealed the presence of IgG antibodies against BP180, linear IgA disease antigen 1 (LAD-1), and laminin α3. The autoimmune bullous disease was suspected, and prednisolone at a concentration of 20 mg/day (0.3 mg/kg/day) was started. When the prednisolone dose was reduced to 10 mg/day, erythema and blisters recurred. The patient was diagnosed with prurigo pigmentosa based on clinical features and was treated successfully with oral doxycycline hydrochloride hydrate and topical tacrolimus ointment. This is the first case of prurigo pigmentosa with blisters in which autoantibodies to the epidermal basement membrane zone were found, which might be secondary non-pathogenic antibodies.
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  • 文章类型: Case Reports
    Checkpoint inhibitors are novel and promising treatment options for different types of cancer. Programmed cell death 1 (PD-1) inhibitors, such as pembrolizumab, have been shown to significantly raise the survival rates of disseminated malignant melanoma (MM). Autoimmune adverse reactions are very common in checkpoint inhibitors. We present 2 cases of bullous pemphigoid, as adverse reactions to pembrolizumab-treated MM.
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  • 文章类型: Case Reports
    Autoimmune bullous diseases are a group of rare, acquired disorders characterized by overlapping features, resistance to treatment, and potential fatality. They need quick and proper management to avoid fatal complications. Ayurveda is found to provide better relief in some autoimmune disorders. Herein, we report a 40-year-old male of autoimmune bullous skin disorder (Visphoṭaka) who failed to respond to allopathic medicines and was subsequently treated with Ayurvedic medicines and achieved complete remission.
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