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Journal of Autoimmunity May 2024Pemphigus, a potentially lethal autoimmune skin disease, is mediated by desmoglein-specific antibodies, manifesting cutaneous and mucosal blisters and erosions. The... (Review)
Review
Pemphigus, a potentially lethal autoimmune skin disease, is mediated by desmoglein-specific antibodies, manifesting cutaneous and mucosal blisters and erosions. The interaction between multiple immune counterparts contributes to the progress of pemphigus. Currently, the emergence of bioinformatic analysis enables investigators to gain a global picture of the pemphigus immune network, based on the exhaustive pedigree annotation of multiple subsets. T helper subsets dominate the landscape as mentioned previously, and innate immune cells have been involved as well. Of particular interests is which phenotype of T cells orchestrates the autoimmune process and chronic inflammation in a certain condition. In this review, the circulatory and peripheral immune cells and cytokine components constituting the immune microenvironment are separately discussed to provide a perspective on pemphigus pathogenesis, with particular reference to insights provided by the bioinformation technique.
PubMed: 38797047
DOI: 10.1016/j.jaut.2024.103261 -
International Immunopharmacology Jun 2024The prevalence and outcomes of coronavirus 2019 (COVID-19) among patients using glucocorticoids and immunosuppressants remain controversial.
BACKGROUND
The prevalence and outcomes of coronavirus 2019 (COVID-19) among patients using glucocorticoids and immunosuppressants remain controversial.
AIM
The study aims to investigate the impact of immunosuppressants especially glucocorticoids on patients in the Autoimmune Bullous Diseases Cohort of West China Hospital (AIBDWCH) during COVID-19.
METHODS
We conducted a cross-sectional survey from December 7, 2022, to February 8, 2023, using questionnaires administered either face-to-face or by phone. COVID-19 cases were classified as confirmed, probable, or suspected according to World Health Organization criteria. Patients were divided into Group A (confirmed and probable cases) and Group B (suspected and other cases). The impact of glucocorticoids and immunosuppressive agents on COVID-19 disease and progression was evaluated with logistic regression models.
RESULTS
This study included 111 patients with pemphigus. Overweight patients had a reduced risk of confirmed COVID-19 (odds ratio [OR] 0.35 [95 % CI 0.13-0.97], p = 0.045). Patients treated with a medium dose of prednisone during the pandemic had a lower incidence of COVID-19 compared to those on low doses, though the difference was not statistically significant. No independent effects of age, sex, comorbidities, and therapies were observed. No significant differences were found in COVID-19 symptoms among different therapy groups.
CONCLUSIONS
Treatment with immunosuppressants, particularly glucocorticoids at low-to-medium doses, did not elevate COVID-19 risk in pemphigus patients. Consistent outcomes across treatments confirm the safety of these therapies during the pandemic.
Topics: Humans; Pemphigus; COVID-19; Male; Female; Middle Aged; Immunosuppressive Agents; Cross-Sectional Studies; Adult; Aged; Glucocorticoids; China; SARS-CoV-2; Surveys and Questionnaires; Risk Factors
PubMed: 38796966
DOI: 10.1016/j.intimp.2024.112307 -
Vaccines Apr 2024Cases of autoimmune bullous dermatosis (AIBD) have been reported following COVID-19 vaccination. (Review)
Review
BACKGROUND
Cases of autoimmune bullous dermatosis (AIBD) have been reported following COVID-19 vaccination.
OBJECTIVE
We aimed to provide an overview of clinical characteristics, treatments, and outcomes of AIBDs following COVID-19 vaccination.
METHODS
We conducted a systematic review and searched the Embase, Cochrane Library, and Medline databases from their inception to 27 March 2024. We included all studies reporting ≥ 1 patient who developed new-onset AIBD or experienced flare of AIBD following at least one dose of any COVID-19 vaccine.
RESULTS
We included 98 studies with 229 patients in the new-onset group and 216 in the flare group. Among the new-onset cases, bullous pemphigoid (BP) was the most frequently reported subtype. Notably, mRNA vaccines were commonly associated with the development of AIBD. Regarding the flare group, pemphigus was the most frequently reported subtype, with the mRNA vaccines being the predominant vaccine type. The onset of AIBD ranged from 1 to 123 days post-vaccination, with most patients displaying favorable outcomes and showing improvement or resolution from 1 week to 8 months after treatment initiation.
CONCLUSIONS
Both new-onset AIBD and exacerbation of pre-existing AIBD may occur following COVID-19 vaccination. Healthcare practitioners should be alert, and post-vaccination monitoring may be essential.
PubMed: 38793716
DOI: 10.3390/vaccines12050465 -
Anais Brasileiros de Dermatologia May 2024Hailey-Hailey disease is a rare genodermatosis described in 1939, with an autosomal dominant inheritance pattern, characterized by compromised adhesion between epidermal...
Hailey-Hailey disease is a rare genodermatosis described in 1939, with an autosomal dominant inheritance pattern, characterized by compromised adhesion between epidermal keratinocytes. It has an estimated prevalence of 1/50,000, with no gender or race predilection. It results from a heterozygous mutation in the ATP2C1 gene, which encodes the transmembrane protein hSPA1C, present in all tissues, with preferential expression in keratinocytes. Mutations in the ATP2C1 gene cause changes in the synthesis of junctional proteins, leading to acantholysis. It usually begins in adulthood, with isolated cases at the extremes of life. It manifests as vesico-bullous lesions mainly in the flexural areas, which develop into erosions and crusts. Chronic lesions may form vegetative or verrucous plaques. Pruritus, a burning feeling and pain are common. It evolves with periods of remission and exacerbation, generally triggered by humidity, friction, heat, trauma and secondary infections. The diagnosis is based on clinical and histopathological criteria: marked suprabasal acantholysis, loosely joined keratinocytes, giving the appearance of a "dilapidated brick wall", with a few dyskeratotic cells. The acantholysis affects the epidermis and spares the adnexal epithelia, which helps in the differential diagnosis with pemphigus vulgaris. Direct immunofluorescence is negative. The main differential diagnoses are Darier disease, pemphigus vegetans, intertrigo, contact dermatitis, and inverse psoriasis. There is no cure and the treatment is challenging, including measures to control heat, sweat and friction, topical medications (corticosteroids, calcineurin inhibitors, antibiotics), systemic medications (antibiotics, corticosteroids, immunosuppressants, retinoids and immunobiologicals) and procedures such as botulinum toxin, laser and surgery. There is a lack of controlled clinical trials to support the choice of the best treatment.
PubMed: 38789364
DOI: 10.1016/j.abd.2023.12.003 -
Journal of the American Academy of... May 2024
JAAD Game Changers: Two decades of using the combination of tetracycline derivatives and niacinamide as steroid-sparing agents in the management of pemphigus: Defining a niche for these low toxicity agents.
PubMed: 38789046
DOI: 10.1016/j.jaad.2024.05.052 -
Cureus Apr 2024Pemphigus, an autoimmune blistering disorder, poses significant therapeutic challenges due to dysregulated B cells and the involvement of CD20. This review assesses the... (Review)
Review
Pemphigus, an autoimmune blistering disorder, poses significant therapeutic challenges due to dysregulated B cells and the involvement of CD20. This review assesses the efficacy of anti-CD20 therapies, including rituximab, ofatumumab, ocrelizumab, and obinutuzumab, in pemphigus treatment. Mechanisms of action, clinical studies, and safety profiles were analyzed, revealing diverse impacts on disease severity. B cell depletion emerged as a pivotal factor, disrupting the autoimmune process and reducing pathogenic antibodies. Varied efficacy and safety profiles among agents underscore the need for personalized treatment strategies guided by biomarkers. Challenges such as resistance and long-term safety concerns necessitate continued research and vigilance. In clinical practice, insights from this review inform nuanced, tailored approaches for improved pemphigus management. The dynamic landscape of emerging therapies and personalized medicine emphasizes the need for ongoing research and strategic clinical decision-making. This review is a foundation for future investigations, providing insights for clinicians and researchers in optimizing pemphigus treatment.
PubMed: 38784354
DOI: 10.7759/cureus.58834 -
The Australasian Journal of Dermatology May 2024
PubMed: 38783774
DOI: 10.1111/ajd.14312 -
Annals of Hematology May 2024Bullous pemphigoid (BP) is a rare blistering disease often considered a primary sign of a paraneoplastic syndrome. Retrospective studies have established its link with...
Bullous pemphigoid (BP) is a rare blistering disease often considered a primary sign of a paraneoplastic syndrome. Retrospective studies have established its link with hematological malignancies, particularly lymphoproliferative disorders. Here, we present what we believe to be the inaugural case of successful simultaneous management of BP and de novo acute myeloid leukemia (AML) in a 28-year-old male patient. Given the rarity and severity of both conditions, our treatment strategy aimed to maximize efficacy by combining immunosuppressive therapy (initially plasmapheresis with high-dose corticosteroids, followed by anti-CD20 monoclonal antibody and intravenous immunoglobulins 2 g/m) with lymphodepleting antileukemic chemotherapy utilizing Fludarabine (FLAG-IDA induction regimen). Following diagnosis, considering the patient's youth and the concurrent presence of two rare and potentially life-threatening diseases, we opted for an aggressive treatment. Upon achieving complete morphological remission of AML with measurable residual disease (MRD) negativity, despite incomplete resolution of BP, we proceeded with high-dose cytarabine consolidation followed by peripheral stem cell harvest and autologous stem cell transplantation (ASCT). Our conditioning regimen for ASCT involved Bu-Cy with the addition of anti-thymocyte globulins. At day + 100 post-ASCT, bone marrow evaluation confirmed morphological remission and MRD negativity. Meanwhile, BP had completely resolved with normalization of BP180 antibody levels.
PubMed: 38780802
DOI: 10.1007/s00277-024-05804-x -
The Journal of Dermatology May 2024
PubMed: 38775209
DOI: 10.1111/1346-8138.17281 -
JAAD International Sep 2024
Review
PubMed: 38774341
DOI: 10.1016/j.jdin.2024.02.011