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Case Reports in Dermatological Medicine 2024Pemphigus foliaceus is an uncommon autoimmune intraepidermal blistering disease characterized by immunoglobulin (Ig) G autoantibodies that attack desmoglein-1 in the...
Pemphigus foliaceus is an uncommon autoimmune intraepidermal blistering disease characterized by immunoglobulin (Ig) G autoantibodies that attack desmoglein-1 in the epidermis. There are two predominant forms of pemphigus foliaceus, sporadic and endemic. Sporadic pemphigus foliaceus is known to be more prevalent in middle-aged and elderly people and to be extremely rare in children. Less than 40 nonendemic pediatric pemphigus foliaceus cases have been documented in the literature. This report documents a case of sporadic pemphigus foliaceus in a 3-year-old Vietnamese girl who presented with generalized scaling and crusted erosions over the body.
PubMed: 38404530
DOI: 10.1155/2024/6748340 -
Alternative Therapies in Health and... Feb 2024Pemphigus vulgaris (PV) accounts for about 80% of all patients with pemphigus, and is the type with the most serious condition and the worst prognosis among autoimmune...
BACKGROUND
Pemphigus vulgaris (PV) accounts for about 80% of all patients with pemphigus, and is the type with the most serious condition and the worst prognosis among autoimmune bullous diseases. Glucocorticoid and immunosuppressor are the main treatment method for PV.
METHODS
The computer retrieves four databases obtain controlled trials on the effects of Rituximab in patients with pemphigus vulgaris. After a rigorous literature quality evaluation, data analysis was performed using RevMan 5.3 software.
RESULTS
7 studies were ultimately included in this meta-analysis. 6 studies reported the Remission rate of the test group and the control group, which was significantly higher (OR:2.26; 95% Cl: 1.80,2.82; P < .01) than the control group. Meta-analysis showed that the improvement of the Recurrence rate was significantly lower than the control group (OR:0.36; 95% Cl: 0.20,0.67; P < .01). Meta-analysis showed that the Adverse reactions was no significant statistical significance than the control group (OR:0.82; 95% Cl: 0.53,1.28; P = .383).
CONCLUSION
The results of this study suggest that Rituximab may be effective in patients with pemphigus vulgaris, which will bring light for patients and doctors. And the above conclusions need to be verified by more high-quality studies.
PubMed: 38401108
DOI: No ID Found -
Medicina (Kaunas, Lithuania) Feb 2024: Rituximab (RTX) has been the predominant treatment for autoimmune bullous diseases (AIBDs). The objective of this research was to assess the advantages and safety...
: 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. : 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. : 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. : 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.
Topics: Humans; Rituximab; Autoimmune Diseases; Pemphigus; Autoantibodies; Desmogleins; Retrospective Studies
PubMed: 38399557
DOI: 10.3390/medicina60020270 -
Veterinary Sciences Feb 2024Canine pemphigus foliaceus (PF) is considered the most common autoimmune skin disease in dogs; the mechanism of PF disease development is currently poorly understood....
Canine pemphigus foliaceus (PF) is considered the most common autoimmune skin disease in dogs; the mechanism of PF disease development is currently poorly understood. Therefore, this study aimed to characterize the molecular mechanisms and altered biological pathways in the skin lesions of canine PF patients. Using an RNA microarray on formalin-fixed, paraffin-embedded samples, we analyzed the transcriptome of canine PF lesional skin ( = 7) compared to healthy skin ( = 5). Of the 800 genes analyzed, 420 differentially expressed genes (DEGs) ( < 0.05) were found. Of those, 338 genes were significantly upregulated, including pro-inflammatory and Th17-related genes. Cell type profiling found enhancement of several cell types, such as neutrophils, T-cells, and macrophages, in PF skin compared to healthy skin. Enrichment analyses of the upregulated DEGs resulted in 78 statistically significant process networks (FDR < 0.05), including the Janus kinase signal transducer and activator of transcription (JAK-STAT) and mitogen-activated protein kinase (MAPK) signaling. In conclusion, canine PF lesional immune signature resembles previously published changes in human pemphigus skin lesions. Further studies with canine PF lesional skin using next-generation sequencing (e.g., RNA sequencing, spatial transcriptomics, etc.) and the development of canine keratinocyte/skin explant PF models are needed to elucidate the pathogenesis of this debilitating disease.
PubMed: 38393106
DOI: 10.3390/vetsci11020089 -
British Dental Journal Feb 2024Pemphigus disease and mucous membrane pemphigoid are autoimmune blistering diseases (AIBDs) which may involve both oral and extra-oral tissues. The Bristol Joint Oral... (Review)
Review
Pemphigus disease and mucous membrane pemphigoid are autoimmune blistering diseases (AIBDs) which may involve both oral and extra-oral tissues. The Bristol Joint Oral Medicine and Dermatology Combined Clinic was set up in 2014, with the primary aim of improving the standard of care for patients with AIBDs. This interdisciplinary approach aimed to address the medical management challenges due to the multisite nature of these AIBDs.We present a narrative report of the clinical work undertaken within this clinic, focused on the management of this patient cohort within a five-year span (2017-2022). This report outlines the multisite nature of AIBDs and the range of topical and systemic treatments that were employed to achieve adequate disease control and optimise outcomes for patients. We reflect on the experiential benefits of this multidisciplinary clinic extended beyond immediate patient benefits to areas such as specialist training, both from a dermatologist's and oral physician's perspective.
Topics: Humans; Dermatology; Mucous Membrane; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Pemphigus; Oral Medicine
PubMed: 38388610
DOI: 10.1038/s41415-024-7074-8 -
SAGE Open Medical Case Reports 2024COVID-19 has been implicated in various cutaneous autoimmune diseases. Pemphigus is a group of autoimmune blistering diseases that target the desmosomal complexes....
COVID-19 has been implicated in various cutaneous autoimmune diseases. Pemphigus is a group of autoimmune blistering diseases that target the desmosomal complexes. Pemphigus triggered by COVID-19 has been seldom reported in the literature and remains both a diagnostic and therapeutic challenge. We report a case of COVID-19-induced pemphigus that responded well to prednisone and mycophenolate mofetil after 9 months from initial presentation. On histologic examination, both intercellular and basement membrane staining were noted. Indirect immunofluorescence staining was positive against the intercellular cement of the stratified epithelium from monkey esophagus. We hypothesize that COVID-19 stimulated the release of multiple pemphigus antigens, which resulted in the unusual histologic pattern reported in the present case. Although malignancy should be suspected when features of paraneoplastic pemphigus, such as basement membrane staining on direct immunofluorescence, are noted, it may also be a histologic pattern of pemphigus secondary to COVID-19 that clinicians may consider.
PubMed: 38371950
DOI: 10.1177/2050313X241231423 -
Indian Journal of Dermatology 2023T helper (Th) cells are involved in the pathogenesis of pemphigus vulgaris (PV). However, the mechanism still needs more exploration.
BACKGROUND
T helper (Th) cells are involved in the pathogenesis of pemphigus vulgaris (PV). However, the mechanism still needs more exploration.
AIMS
This study aimed to evaluate the molecular mechanism of the dysregulation of Th17 cells in the peripheral blood of patients with PV.
MATERIALS AND METHODS
Serum levels of IL-17 and anti-Dsg3 titres in patients with PV were analysed using ELISA. The mRNA expression of retinoic acid orphan receptor γt (RORγt) in CD4 T cells was detected using reverse transcription-quantitative PCR (qPCR). The number of Th17 cells was examined using flow cytometry. Western blot analysis and immunofluorescent staining were also performed to investigate the expression levels of ERK/MAPK signalling proteins and Th17 lineage-associated proteins.
RESULTS
The proportion of Th17 cells and Th17 spectrum-associated proteins (p-STAT3, RORγt and IL-17) were upregulated in CD4+ cells in PV patients. The increased transcriptional levels of RORγt and IL-17 correlated positively with the severity of PV. Elevated phosphorylation of the ERK signalling factors was found in the collected CD4+ T cells in PV patients. The inhibition of the ERK signalling pathway significantly reduced the differentiation of Th17 cells in PV patients .
CONCLUSION
Th17 cells are essential in the dysregulation of PV, and ERK signalling is involved in Th17-type immunity and promotes the development of PV. The study here provides us with a potential therapeutic target for PV.
PubMed: 38371571
DOI: 10.4103/ijd.ijd_924_22 -
Indian Journal of Dermatology 2023
PubMed: 38371552
DOI: 10.4103/ijd.ijd_1061_20 -
SAGE Open Medical Case Reports 2024Pemphigoid vegetans is a rare variant of bullous pemphigoid. Only 13 cases have been documented in the medical literature. The lesions clinically resemble pemphigus...
Pemphigoid vegetans is a rare variant of bullous pemphigoid. Only 13 cases have been documented in the medical literature. The lesions clinically resemble pemphigus vegetans, but the histology is consistent with bullous pemphigoid. We present the case of a 63-year-old woman with painful vesicular and vegetative lesions progressing for 4 months. Histopathology showed a dermal-epidermal cleavage, epidermal acanthosis and an eosinophilic infiltrate. Direct immunofluorescence showed linear deposition of IgG and IgA at the junctional level. The diagnosis of pemphigoid vegetans was retained and successfully treated with oral corticosteroid, dapsone and mycophenolate mofetil.
PubMed: 38362225
DOI: 10.1177/2050313X241231535