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.

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Patients with autoimmune bullous diseases (AIBDs) are considered to be immunocompromised and, consequently, they may be more susceptible to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and have poorer outcomes. However, the risk and repercussions of SARS-CoV-2 infection in patients with AIBDs have not been fully understood. Therefore, we aimed to investigate the risk factors of SARS-CoV-2 infection and the impact of SARS-CoV-2 on patients with AIBDs. From December 2022 to January 2023, all patients with AIBDs who visited our clinic were enrolled in this study. Meanwhile, web-based questionnaires and telesurveys were used as supplements. Information about patients\' demographics, comorbidities, SARS-CoV-2 infection, and vaccination, as well as AIBD status and treatments were collected and analyzed. The diagnosis of SARS-CoV-2 infection was based on a positive polymerase chain reaction test, and/or an antigen test, or the presence of typical symptoms in conjunction with an epidemiological history. Finally, 95 patients with AIBDs were enrolled, including 47 cases of pemphigus and 48 cases of pemphigoid cases, and 73 had symptoms consistent with coronavirus disease 2019. Common symptoms after SARS-CoV-2 infection were fever (80.8%), fatigue (75.0%), cough (71.2%), muscle/joint pain (49.3%), and sore throat (45.2%). No significant differences were found between SARS-CoV-2-infected and asymptomatic patients. Patients who had hypertension (p = 0.034), hyperlipidemia (p = 0.017), or more than two comorbidities (p = 0.011) were more likely to develop pneumonia after infection. Patients with pemphigus who did not achieve disease control (p = 0.045) or had an oral corticosteroid dose ≥15 mg/day (p = 0.024) and patients with pemphigoid with a disease duration ≥2 years (p = 0.037) were more prone to AIBDs aggravation. In conclusion, patients with AIBDs are generally susceptible to SARS-CoV-2 infection. Individuals with newly diagnosed AIBDs, uncontrolled disease, and a higher corticosteroid dose are more susceptible to disease exacerbation.

Psoriasis is an immune-mediated chronic inflammatory disease that can be combined with complications such as diabetes, cardiovascular disease, obesity, and kidney disease. The comorbidity of psoriasis with autoimmune bullous diseases (AIBD) has been reported previously in several cases, the most frequent of which is bullous pemphigoid (BP). The underlying mechanisms of psoriasis with BP are not clear and there are no uniform treatment criteria. Based on previous case reports, the coexistence of psoriasis and BP may be related to inflammatory activity, medications, phototherapy, and infection. We report a case of a psoriasis patient who developed BP after taking Chinese herbal compounds and was successfully treated with dupilumab, which is the first reported case of applying dupilumab to treat psoriasis with BP comorbidities.

Autoimmune bullous diseases (AIBDs), which are a group of tissue-specific autoimmune diseases of the skin, present with various blistering lesions on the skin and mucous membranes, and show autoantibodies of IgG, IgA and IgM against epidermal cell surfaces and basement membrane zone. To date, AIBDs have been classified into a number of distinct subtypes by clinical and histopathological findings, and immunological characteristics. In addition, various biochemical and molecular biological studies have identified various novel autoantigens in AIBDs, which has resulted in proposals of new subtypes of AIBDs. In this article, we summarized various distinct AIBDs, and proposed the latest and most comprehensive classification of AIBDs with their autoantigen molecules.

BACKGROUND: Bullous pemphigoid (BP) mostly involves elderly patients. The diagnosis of BP requires special immunological tests, which makes some patients unable to be diagnosed and treated timely.
OBJECTIVE: The accuracy and application value of immune colloidal gold technique (ICGT) in BP were evaluated. The colloidal gold was conjugated with recombinant BP180 NC16A protein and mouse IgG antibody. As the test and control lines, the mouse-anti-human IgG and goat-anti-mouse IgG, respectively, were blotted on the nitrocellulose membrane.
METHODS: 414 serum samples of consecutive patients with suspected BP and 15 samples from healthy donors were recruited. The consistency between ICGT and ELISA, and between serum and plasma/whole blood were evaluated. Subgroup analyses were performed in terms of clinical characteristics. We also followed up 65 BP patients\' strip results to explore the predictive value of ICGT.
RESULTS: Strong agreements between ICGT and ELISA(κ = 0.902) and between plasma/whole blood and serum samples (κ = 0.980) with good stability were observed. The ICGT achieved sensitivity of 93.9%, and specificity of 97.6%. In subgroup analysis, the sensitivity was significantly higher in older patients (96.3%), and with more typical lesions such as blisters (96.2%) and erosions (92.4%). In follow-up, we also found BP patients who kept ICGT-negative in remission state all got consecutive positive strips 1-3 weeks prior to mild new activity or flare.
CONCLUSIONS: ICGT shows high potential as a rapid and stable option for the diagnosis and monitoring of BP. Further investigations are needed to re-evaluate this technique in a prospective study with a multicenter design.

Common autoimmune bullous diseases (AIBDs) include pemphigus and bullous pemphigoid (BP), which are primarily caused by IgG autoantibodies against the structural proteins of desmosomes at the cell-cell junction and hemidesmosomes at the epidermal-dermal junction. Few studies have assessed nail changes in patients with pemphigus or BP. In the present study, we collected the clinical data of 191 patients with AIBDs (108 patients with pemphigus and 83 patients with BP) and 200 control subjects. Nail changes were observed in 77.0% (147/191), 77.8% (84/108), and 75.9% (63/83) of patients with AIBDs, pemphigus, and BP, respectively, and 14.5% (29/200) of control subjects. Beau\'s lines and paronychia were the most common nail involvement, observed in 22.5% (43/191) and 22.5% (43/191) of patients with AIBDs, 25.0% (27/108) and 25.9% (28/108) of patients with pemphigus, 19.3% (16/83) and 18.1% (15/83) of patients with BP, respectively. The autoimmune bullous skin disorder intensity score (ABSIS) and the onset time of patients with pemphigus or BP with nail changes were different. Onychomycosis accounted for 21.5% (41/191) of all patients with AIBDs. The ABSIS was correlated with nail involvement in patients with BP (r = 0.46, p < 0.001), and weakly correlated with nail involvement in patients with AIBDs (r = 0.37, p < 0.001), pemphigus (r = 0.29, p = 0.009), and pemphigus vulgaris (PV; r = 0.35, p = 0.008). No correlation was observed between nail involvement and disease antibody titers. In conclusion, nail changes are frequently observed in patients with pemphigus and BP. The type and onset time of nail changes may indicate the severity of pemphigus and BP, which warrants the attention of dermatologists.

Pemphigus is a rare autoimmune blistering disease, involving potentially life-threatening conditions often requiring immunosuppression. Currently, the COVID-19 pandemic caused by severe acute respiratory disease coronavirus 2 (SARS-CoV-2) infection has become a global public emergency. Vaccines are the most effective defense against COVID-19 infection. However, in clinic, there are cases of new onset or flare of pemphigus following COVID-19 vaccination, where vaccines have manifested significantly desirable risk-benefit profiles for patients. Although Rituximab, as first-line therapy, may impair humoral immunity, pemphigus may not predispose to develop COVID-19 infection compared to a healthy population. Conversely, delay or interruption of immunosuppressants probably results in unfavorable clinical outcomes for disease progression. Overall, clinicians should encourage their patients to undergo the vaccination after a comprehensive assessment. The definite association between COVID-19 vaccination and pemphigus remains to be further elucidated. Herein, we provide an overview of the published studies to date on COVID-19 and pemphigus as well as the exploration of their complicated interplay. In addition, we discuss the management strategies for pemphigus patients in this special period, in an effort to more effectively establish a standard treatment paradigm for this particular patient group.

BACKGROUND: Autoimmune blistering diseases (AIBDs) are a group of fatal diseases with specific autoantibodies. BIOCHIP mosaic is a novel and all-in-one measure used for the rapid diagnosis of AIBDs.
OBJECTIVE: To evaluate the diagnostic accuracy based on BIOCHIP mosaic (FA1501-1005-60) in Chinese patients with AIBDs.
METHODS: Seventy-seven patients with AIBDs and 20 controls were enrolled. The BIOCHIP mosaic was performed using both serum and plasma samples.
RESULTS: Based on BIOCHIP mosaic, the data from paired plasma and serum samples demonstrated a high degree of concordance (Cohen\'s kappa = 0.896-1.000) for autoantibodies against Dsg1, Dsg3, BP180-NC16A-4X, BP230gC, prickle-cell desmosomes, and pemphigoid antigens. Moreover, BIOCHIP mosaic also demonstrated a high degree of consistency for the detection rate of anti-Dsg1, Dsg3, plakins, BP180-NC16A-4X and non-collagenous domain of type VII collagen autoantibodies for the diagnosis of pemphigus foliaceus (77.3%), pemphigus vulgaris (88.6%), paraneoplastic pemphigus (100.0%), bullous pemphigoid (92.8%) and epidermolysis bullosa acquisita (99.0%), respectively.
CONCLUSIONS: Using BIOCHIP mosaic, serum and plasma samples may be used interchangeably at 1/10 dilution. Overall, the BIOCHIP mosaic was shown to be a useful and accurate tool for the diagnosis of AIBDs.

Since late December 2019, an outbreak of the novel coronavirus disease, which is mainly characterized by pulmonary lesions, has spread quickly worldwide. With the spread of the novel coronavirus, the outpatient lines of hospitals have mostly shut down, which means that routine clinical diagnosis and treatment for autoimmune bullous diseases patients have been disturbed. Due to the serious condition of autoimmune bullous diseases patients, they are prone to immune suppression and circulatory failure, and are more susceptible to infection than healthy individuals. These patients should thus be a priority group for novel coronavirus disease prevention. In this context, the protection and control measures for autoimmune bullous diseases patients against the novel coronavirus are of serious concern. Clinicians should strengthen their communication with patients, pay attention to changes in patients\' conditions, and carefully adjust the treatment strategy, while protecting against the novel coronavirus infection, to ensure the continuity, safety, and effectiveness of autoimmune bullous diseases treatment.