Epidermolysis bullosa acquisita treated with rituximab

Mendes, Sónia Raquel; Coutinho, Inês; Cardoso, José Carlos

doi:10.1136/bcr-2021-243432

Cited by 7 publications

(5 citation statements)

References 5 publications

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“…This observation is supported by the reports of 29 EBA cases treated with IVIG summarized by Koga et al (23). Twenty-six of these cases achieved at least partial remission, and only three patients did not benefit from IVIG infusions (13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31). In Case 2, IVIG infusions were found to be sufficient treatment, while in Case 3, additional treatment with rituximab was necessary.…”

Section: Ivig In Eba Treatmentsupporting

confidence: 52%

Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review

et al. 2023

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Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.

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Section: Ivig In Eba Treatmentsupporting

confidence: 52%

Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review

et al. 2023

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“…Rituximab (RTX) has shown potential as an alternative treatment, with some success reported in a small numbers of cases. 2,3 Herein, we report on the efficacy, safety, and treatment durability of RTX in the largest series of patients with EBA.…”

mentioning

confidence: 99%

Rituximab for the treatment of epidermolysis bullosa acquisita: A large case series of 15 patients

Dasdar

Kianfar

Tavakolpour

et al. 2023

J Deutsche Derma Gesell

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“…EBA is a rare dermatosis with often unsatisfactory treatment outcomes. Evidence suggests that RTX holds promise as an efficacious therapeutic intervention, substantiated by documented successes in a limited number of patient cases within the existing literature [30,31]. A patient enrolled in our study cohort, diagnosed with EBA, exhibited initial resistance to RTX intervention.…”

Section: Discussionmentioning

confidence: 84%

Rituximab in the Management of Autoimmune Bullous Diseases: A Treatment-Resistant Case Series from a Single Central European Referral Center

Spałek,

Jałowska,

Bowszyc-Dmochowska

et al. 2024

Medicina

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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.

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Epidermolysis bullosa acquisita treated with rituximab

Cited by 7 publications

References 5 publications

Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review

Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review

Rituximab for the treatment of epidermolysis bullosa acquisita: A large case series of 15 patients

Rituximab in the Management of Autoimmune Bullous Diseases: A Treatment-Resistant Case Series from a Single Central European Referral Center

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