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The British Journal of Dermatology Jun 2024Pemphigus vulgaris (PV) is characterized by autoantibodies targeting keratinocytes adhesion proteins desmoglein (Dsg) 1 and 3, and by the HLA-DRB1-0402 predisposition...
BACKGROUND
Pemphigus vulgaris (PV) is characterized by autoantibodies targeting keratinocytes adhesion proteins desmoglein (Dsg) 1 and 3, and by the HLA-DRB1-0402 predisposition allele. Treatment using rituximab (RTX) combined with short-term corticosteroids (CS) allows disease control and long-lasting remission.
OBJECTIVE
The principal aim of this study is to evaluate the impact of RTX on the circulating subpopulations of Dsg-3-specific T lymphocytes that specifically regulate B cell responses: follicular helper (Tfh) and follicular regulatory T (Tfr) lymphocytes.
METHODS
Using the HLA-DRB1-0402 tetramer loaded with the Dsg-3 immunodominant peptide, we analysed by flow cytometry the frequency, the polarisation and the activation status of blood Dsg-3-specific follicular T cell populations at baseline, Month 6 and long-term follow-up (Month 60-90) from PV patients.
RESULTS
At baseline, we observed a predominance of Tfh1* and Tfh17 subsets and an underrepresentation of the Tfh2 subset among autoreactive Dsg-3-specific Tfh cells as compared with non-autoreactive Tfh cells. RTX treatment induced a decrease of autoreactive Tfh cells with no effect on their polarisation during patients' follow-up. In parallel, we observed the emergence of a Dsg-3-specific Tfr subpopulation with a significant overexpression of the surface activation markers PD1, ICOS, and CD25 that was not observed at the surface of autoreactive Tfh and non-autoreactive Tfr cells of the same PV patients. In contrast, a very few Dsg-3 specific Tfr cells were observed in PV patients treated with CS alone.
CONCLUSION
Here we show that the emergence of circulating autoreactive Dsg-3-specific Tfr cells is associated with the long-term efficacy of RTX in PV patients.
PubMed: 38848544
DOI: 10.1093/bjd/ljae220 -
Indian Dermatology Online Journal 2024
PubMed: 38845638
DOI: 10.4103/idoj.idoj_351_23 -
Indian Dermatology Online Journal 2024Rituximab infusion and dexamethasone-cyclophosphamide pulse (DCP) are the two most popular regimens used in pemphigus vulgaris (PV) in India.
Comparison of the Clinical Efficacy of Rituximab Infusion and Dexamethasone-Cyclophosphamide Pulse Therapy and Their Effect on Serum Th1, Th2, and Th17 Cytokines in Pemphigus Vulgaris-A Prospective, Nonrandomized, Comparative Pilot Study.
BACKGROUND
Rituximab infusion and dexamethasone-cyclophosphamide pulse (DCP) are the two most popular regimens used in pemphigus vulgaris (PV) in India.
OBJECTIVE
The present study compared the clinical efficacy of rituximab and DCP in Indian PV patients and their effects on serum Th1,2, and 17 cytokine levels.
MATERIALS AND METHODS
A total of 37 patients received DCP (Group A, = 22) or rituximab (Group B, rheumatoid arthritis protocol ( = 15)) as per patients' preference. They were monitored for clinical response, adverse events (AEs), changes in serum anti-desmoglein-1,3 antibody titers and Th1,2 and 17 cytokine levels at baseline and weeks 20 and 52.
RESULTS
The proportion of patients attaining disease control, remission, and relapse in groups A and B were 82% and 93%; 73% and 93%; and 27% and 50%, respectively, after a median duration of 2 months each for disease control; 4 and 4.5 months for remission; and 5 and 7 months for relapse post remission. The musculoskeletal AEs were the highest in the two groups. Significant and comparable decreases in anti-dsg1 and 3 titers from baseline to weeks 20 and 52 were observed in both groups. Th1 and Th17 cytokine levels decreased, while Th2 cytokines increased post-treatment in both groups. However, no correlation was found between change in body surface area of involvement by PV and anti-dsg titers and cytokine levels before and after therapy in both groups.
CONCLUSION
Comparable clinical efficacy between DCP and rituximab was observed.
PubMed: 38845632
DOI: 10.4103/idoj.idoj_558_23 -
Indian Journal of Dermatology,... Apr 2024Background Owing to the lack of standardised audio-visual (A-V) instructions to take photographs, patients with pemphigus faced difficulties during tele-consultation in...
Construction and validation of an audio-visual tool to instruct caregivers of patients with pemphigus in photographing skin and oral cavity lesions during tele-consultation: A cross-sectional study.
Background Owing to the lack of standardised audio-visual (A-V) instructions to take photographs, patients with pemphigus faced difficulties during tele-consultation in COVID-19 pandemic. Objective To construct and validate an A-V instruction tool to take photographs of skin and oral cavity lesions of pemphigus. Methods In this observational study, we included patients with pemphigus of either gender seeking tele-consultation, aged 18 years or older. A-V instructions demonstrating skin and oral cavity photography were designed and shared with the patients. They were requested to send pictures of lesions that complied with the instructions. They were then required to complete a 10-item questionnaire for face validation in the two following rounds. The videos were content validated by 14 experts in the field of clinical dermatology. Results Forty-eight patients took part in face validation. A majority of patients, 47 (97.9%) and 45 (93.8%); rated the audio and video quality as being above average, respectively. Forty-seven patients (97.9%) said the instructional videos were useful, and 42 patients (87.5%) said they did not need to take any further images to show how severe their disease was. The average scale content validity index for the instructions on skin imaging and the oral cavity imaging during round 1 of content validation was 0.863 and 0.836, respectively. Limitation Validated instruction videos are in Hindi language and need to be further translated and validated in other local languages for use in non-Hindi speaking regions. Conclusion A-V instructions were useful to take photographs during tele-consultation.
PubMed: 38841977
DOI: 10.25259/IJDVL_361_2023 -
Indian Journal of Dermatology,... May 2024
PubMed: 38841926
DOI: 10.25259/IJDVL_481_2024 -
Indian Journal of Dermatology 2024Gluten, a polypeptide hapten, found in many cereals such as barley, wheat, rye, oats, and others, has been recently implicated in a range of cutaneous disorders ranging... (Review)
Review
Gluten, a polypeptide hapten, found in many cereals such as barley, wheat, rye, oats, and others, has been recently implicated in a range of cutaneous disorders ranging from chronic plaque psoriasis through psoriatic arthritis, urticaria (chronic as well as paediatric onset), and angioedema to lichen planus, vitiligo, and rosacea. The evidence for them is still not well reviewed. To generate evidence for the causal role of gluten in various dermatological disorders. The Pubmed, MedLine, and EMBASE databases were searched using the keywords "Gluten" and one of the dermatoses, namely, "Atopic Dermatitis", "Vasculitis", "Psoriasis", "Psoriatic Arthritis", "Acne", "Alopecia Areata", and "Immunobullous disorders". All articles published in English for which free full text was available were taken into consideration. The search strategy returned in a total of 1487 articles which were screened for relevance and elimination of duplicates. Ultimately, around 114 articles were deemed suitable. The data were extracted and presented in the narrative review format. A simple and cost-effective solution to many of these chronic and lifelong conditions is to restrict gluten in the diet. However, the dermatologist would do well to remember that in the vast majority of dermatological disorders including the ones listed here, gluten restriction is not warranted and can even lead to nutritional deficiencies. The evidence varied from Grade I for some disorders like psoriatic arthritis to Grade IV to most disorders like acne, vitiligo, vasculitis, and atopic dermatitis. Herein, we review the evidence for each of these conditions and make practical recommendations for gluten restriction in them.
PubMed: 38841247
DOI: 10.4103/ijd.ijd_815_22 -
Indian Journal of Dermatology 2024
PubMed: 38841239
DOI: 10.4103/ijd.ijd_1101_23 -
Veterinary Dermatology Jun 2024Cytological detection of acantholytic keratinocytes (acantholytic cells [AC]) helps to identify canine pemphigus foliaceus (cPF) yet AC also occurs in superficial...
BACKGROUND
Cytological detection of acantholytic keratinocytes (acantholytic cells [AC]) helps to identify canine pemphigus foliaceus (cPF) yet AC also occurs in superficial pyoderma (SP), the main differential diagnosis.
HYPOTHESIS/OBJECTIVES
To compare selected cytomorphological features of cPF and SP and to establish cytological diagnostic criteria that could differentiate cPF from SP.
ANIMALS
40 and 51 client-owned dogs with PF and SP, respectively.
MATERIALS AND METHODS
Impression smears from cPF (64), impetigo (40) and exfoliative superficial pyoderma (ESP) (17) samples were stained with Romanowsky stain, randomised, blinded and evaluated by two investigators independently. The entire sample was screened (×500 or ×1000 magnification) for round (AC1), boat (AC2) and raft AC, eosinophils and bacteria. Interobserver agreements were calculated.
RESULTS
The average number of the 10 highest ×500 fields for AC1 and AC2 was significantly higher in PF than SP (p < 0.0001; Kruskal-Wallis test). Rafts and eosinophils were more common in PF than SP (p < 0.0001; chi-square test), while bacteria were rare in PF (5%; p < 0.0001; chi-square test). Observations between the experienced and novice investigators were highly correlated. An ROC analysis identified five AC1/×500-magnification field as a suitable cut-off value for predicting PF diagnosis. This cut-off value was tested by two additional investigators, who identified sensitivity of 84%-100%, specificity of 95%-97% and accuracy of 95%-96% for the diagnosis of cPF.
CONCLUSIONS AND CLINICAL RELEVANCE
Criterion-based impression smear cytological evaluation can provide strong evidence to support the clinical diagnosis. Acantholytic cell morphology varies in cPF and SP, and experience can improve accuracy in cytological differentiation.
PubMed: 38840462
DOI: 10.1111/vde.13267 -
BMJ Case Reports Jun 2024Paraneoplastic pemphigus (PNP) is a rare disease with an unclear mechanism of pathogenesis. We present a case of a male patient who presented with wound management after...
Paraneoplastic pemphigus (PNP) is a rare disease with an unclear mechanism of pathogenesis. We present a case of a male patient who presented with wound management after being diagnosed with Castleman disease-associated paraneoplastic pemphigus (PNP). The patient's condition was not improving; as a result, extensive workup was repeated, which confirmed the diagnosis of aggressive T cell lymphoblastic lymphoma. Our case signifies the importance of keeping a high index of suspicion for PNP-associated malignancies. This case report also adds emphasis to the diagnostic challenges faced by clinicians, making clinical correlation with multidisciplinary approach essential. Therefore, if clinically indicated, we need to revisit the diagnosis and seek alternative explanations to prevent delays in management.
Topics: Humans; Pemphigus; Male; Paraneoplastic Syndromes; Castleman Disease; Diagnosis, Differential; Middle Aged
PubMed: 38839409
DOI: 10.1136/bcr-2023-258580 -
Journal of the European Academy of... Jun 2024Autoimmune blistering diseases (AIBDs) are severe dermatologic disorders known for their debilitating physical impact. Recent research has reported that AIBDs lead to...
BACKGROUND
Autoimmune blistering diseases (AIBDs) are severe dermatologic disorders known for their debilitating physical impact. Recent research has reported that AIBDs lead to psychosocial impairment, including depression and anxiety. Missing from the extant literature is an examination of the impact of AIBDs on body image and related psychological constructs.
OBJECTIVES
The current study seeks to characterize the psychological and social consequences of AIBD diagnosis, with particular attention to body image dissatisfaction.
METHODS
We conducted a survey study of adults with AIBDs. The survey was open from February 2023 to March 2023. Validated self-report questionnaires assessed depressive symptomatology, body image disturbance and quality of life. Demographic information and self-reported psychiatric history before and after AIBD diagnosis were collected via self-report. Participants were 451 adults with AIBDs, recruited through the International Pemphigus and Pemphigoid Foundation newsletters, email distribution lists and social media.
RESULTS
Participants reported increased incidence of psychiatric disorders following AIBD diagnosis. Participants reported high levels of depressive symptomatology and impairments to quality of life compared to other patient groups. The sample reported extremely high levels of body image disturbance, more so than other patients with disfiguring diseases or injury. Correlation analyses revealed significant relationships between body image variables and quality of life, even after controlling for depression.
CONCLUSIONS
Current treatment guidelines for AIBDs focus primarily on the management of disease flares and the consequences of immunosuppression, without consideration of the psychosocial consequences of the disease. The current study underscores the need for mental health support for patients with AIBDs.
PubMed: 38837452
DOI: 10.1111/jdv.20156