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Dermatology Practical & Conceptual Apr 2024Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa.
Evaluation of the Quality of Life and the Demographic and Clinical Characteristics of Patients With Pemphigus With Oral Mucosal İnvolvement: A Multicenter Observational Study.
INTRODUCTION
Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa.
OBJECTIVES
This study aimed to determine the demographic, clinical and treatment characteristics of PV patients with oral mucosal involvement and to assess the impact on their quality of life.
METHODS
We conducted a prospective observational study among 106 patients diagnosed with PV and presenting oral mucosal involvement. Demographic data, clinical and treatment characteristics, and quality of life questionnaires were recorded.
RESULTS
The study included 106 patients, 55 (51.89%) were male and there was a predominance of the mucocutaneous subtype in 83 individuals (78.38%). Oral mucosa was the initial site of manifestation in 44 patients (41.51%). Bilateral buccal mucosa was the most frequently affected site. The predominant symptom reported was a burning sensation, noted in 91 patients (85.85%). Oral mucosal examination revealed erosions in 85.85% of the patients. Systemic steroids were the most commonly administered treatment, and rituximab was used in 18 patients (16.98%). A positive and significant correlation was found between pemphigus severity and Oral Health Impact Profile-14, Dermatology Life Quality Index and Dermatological Quality of Life Scale scores (P < 0.05). The presence of superficial ulcers, flaccid bullae, lesion diameter ≥1 cm, and >10 lesions were factors that markedly diminished quality of life. Complete response to treatment was noted in all patients administered rituximab.
CONCLUSIONS
The most common area of involvement was bilateral buccal mucosa, and the severity of PV closely correlated with a decline in quality of life measures. These results highlight the need for careful clinical oversight of PV, taking into account its effects on patients quality of life.
PubMed: 38810063
DOI: 10.5826/dpc.1402a99 -
Archives of Dermatological Research May 2024
Topics: Humans; Pemphigus; Gastrointestinal Microbiome; Mendelian Randomization Analysis; Polymorphism, Single Nucleotide
PubMed: 38809337
DOI: 10.1007/s00403-024-02934-0 -
International Journal of Dermatology May 2024
PubMed: 38807277
DOI: 10.1111/ijd.17275 -
Dermatology Research and Practice 2024Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially life-threatening mucocutaneous blistering diseases that clinically can...
BACKGROUND
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially life-threatening mucocutaneous blistering diseases that clinically can resemble autoimmune bullous diseases. Moreover, it has been shown that autoantibodies against epidermal proteins are present in SJS/TEN.
OBJECTIVES
To establish the presence of antibodies against desmosomal and hemidesmosomal proteins in confirmed SJS/TEN patients.
METHODS
Serum of SJS/TEN patients diagnosed based on clinical criteria, e.g., epidermal detachment with erosions and severe mucosal lesions, (suspicion of) a culprit drug, and matching histologic results was evaluated by various techniques, e.g., indirect immunofluorescence on monkey esophagus, salt split skin and rat bladder, immunoblotting (IB) and immunoprecipitation (IP), ELISAs against desmogleins and BP180, keratinocyte footprint assay, and keratinocyte binding assay.
RESULTS
A total of 28 patients were included in this study, 15 men and 13 women with a mean age of 56 years. In most patients, none of the serological tests were positive. In two patients, an elevated DSG3 titer was found suspicious for pemphigus vulgaris. Three patients had elevated NC16a titers, suggesting bullous pemphigoid. However, in all these patients, no other tests were positive and in these patients, the biopsy for direct immunofluorescence showed no evidence for an autoimmune bullous disease. Three patients showed reactivity against rat bladder rat bladder; these were, however, completely negative for A2ML1, envoplakin, and periplakin in the IB as well as the IP.
CONCLUSIONS
Serological analysis for desmosomal and hemidesmosomal antibodies is reliable to rule an autoimmune bullous disease in patients with suspected SJS/TEN. However, one should not rely on one single test method since false positive results can occur. Moreover, this study also makes it less plausible that antibodies against desmosomal and/or hemidesmosomal components are involved in the pathogenesis of SJS/TEN.
PubMed: 38803350
DOI: 10.1155/2024/5504462 -
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