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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.

OBJECTIVE: Autoimmune bullous diseases, connective tissue diseases, and vasculitis represent a group of severe rare skin diseases. While glucocorticoids and immunosuppressive agents serve as standard treatments for these diseases, their efficacy is limited due to adverse side effects, indicating the need for alternative approaches. Biologics have been used in the management of some rare skin diseases. However, the use of biologics is associated with concerns, such as infection risk and high costs, prompting the quest for efficacious and cost-effective alternatives. This study discusses the safety issues associated with tofacitinib and its potential in treating rare skin diseases.
METHODS: This narrative review focuses on the pharmacodynamic properties of tofacitinib and its impact on the JAK/STAT pathway. In addition, we present a comprehensive discussion of the effects and mechanism of action of tofacitinib for each severe rare skin disease.
RESULTS: This role of tofacitinib in treating severe rare skin diseases has been discussed, shedding light on its promising prospects as a treatment modality. Few reports of serious adverse events are available in patients treated with tofacitinib.
CONCLUSIONS: We explored the mechanism of action, efficacy, and safety considerations of tofacitinib and found that it can be used as a treatment option for rare skin diseases. However, multicenter clinical studies are needed to confirm the efficacy and safety of JAK inhibitors.

As part of its therapeutic patient education program, the Avicenne hospital in the Paris region invites patients with an autoimmune bullous disease to a workshop dedicated to local skin and mucous membrane care. Together, a nurse, patient partners and patients review best practices in hygiene care and treatment of the skin, eyes, nose, mouth, genitals and anus. This is essential for healing lesions and avoiding local complications.

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Introduction The follow-up of patients with autoimmune bullous diseases (AIBDs) was temporarily interrupted during the initial phase of the COVID-19 pandemic due to restrictions in healthcare services, given the high contagiousness and rapid spread of SARS-CoV-2. Our objective was to assess the impact of the initial phase of the COVID-19 pandemic on the treatments and disease activity of AIBD patients. Methods We conducted a telephone survey of patients with AIBDs who had been regularly followed up in our hospital prior to the onset of the pandemic. A structured questionnaire that we designed was used. This questionnaire comprised questions examining the following issues between March and June of 2020: patients\' follow-up, treatment, COVID-19 infection status, and changes in disease activity. Results Thirty-nine patients were included in the study. Among those, 26 (66.7%) were immunosuppressed. The frequency of follow-up for 37 patients (94.9%) changed significantly (p<0.001): 28 patients (71.8%) did not visit the hospital, and 26 of them (92.9%) did not communicate at all. The treatment for 10 patients (25.6%) was altered, either by their physician or by themselves. Disease activity reactivated in patients who altered their own treatments. There was only one patient (2.6%) who contracted COVID-19. Conclusions Documenting this period revealed that some patients were negatively impacted by the pandemic initially. The most significant contributing factor was the interruption of patient-physician communication.

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Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists.

UNASSIGNED: Treatment options for autoimmune bullous diseases (AIBD) are currently limited to corticosteroids and traditional immunomodulants and immunosuppressants that are associated with unfavorable adverse effect profiles. The most frequent AIBDs, i.e. bullous pemphigoid, pemphigus vulgaris, and mucous membrane pemphigoid, impose a high disease burden onto affected patients and can be detrimental due to infections, exsiccosis, and impaired food intake. Significant progress has been made in elucidating disease mechanisms and key mediators by in vivo and in vitro models, thus identifying a multifaceted range of possible drug targets. However, except for rituximab for pemphigus vulgaris, no new drugs have been approved for the treatment of AIBDs in the last decades.
UNASSIGNED: This review covers new drug developments and includes ongoing or completed phase 2 and 3 clinical trials. Studies were identified by querying the registries of ClinicalTrials.gov and Cochrane Library.
UNASSIGNED: Promising results were shown for a variety of new agents including nomacopan, efgartigimod, omalizumab, dupilumab, as well as chimeric autoantibody receptor T cells. Clinical translation in the field of AIBDs is highly active, and we anticipate significant advances in the treatment landscape.

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.