Evaluation of desmoglein 1 and 3 autoantibodies in pemphigus vulgaris: correlation with disease severity

Delavarian, Zahra; Layegh, Pouran; Pakfetrat, Atessa; Zarghi, Nazila; Khorashadizadeh, Mahboubeh; Ghazi, Ala

doi:10.4317/jced.56289

Cited by 12 publications

(13 citation statements)

References 21 publications

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“…A previous study suggested that anti-desmoglein-1 autoantibody titers seem to be more useful in showing the extent of the disease and pemphigus activity with mucocutaneous lesions. In contrast, antidesmoglein-3 autoantibody titers can be used for diagnostic purposes [21].…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of oral pemphigus vulgaris in Thai patients

Kaomongkolgit¹,

Sarideechaigul²,

Klanrit³

et al. 2021

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Background. Pemphigus vulgaris (PV) is a rare, life-threatening autoimmune disease and varies in its clinical profile and epidemiology in different regions of the world.Objectives. This study aimed to evaluate the demographic and clinical characteristics of oral PV patients in the lower north and northeast of Thailand. Material and methods. A retrospective study of PV patients who visited the Oral Medicine Clinic, Dental Hospital, Naresuan University and Khon Kaen University during the 2010-2020 period. Results. 21 oral PV patients were included. Among these, 16 (76.2%) were female, and 5 (23.8%) were male, with a female to male ratio of 3.2:1. The mean age of the patients was 47.7 ± 14.2 (range 25 to 74) years. 8 patients (38.1%) had a history of systemic diseases, and hypertension was predominant. The mean duration of the disease was 6 ± 3.42 (range 1 to 12) months. The most common complaint at presentation was oral ulceration with a burning sensation. The most affected sites were the buccal mucosa and gingiva (80.9%). Extraoral involvement was found in 14 patients (66.7%). Patients who had oral lesions before skin lesions found the mean duration was 4.63 ± 5.13 (range 1 to 16) months. Most of the patients (61.9%) were prescribed a combination of topical and systemic steroids. Conclusions. The present study illustrates the demographics and clinical characteristics of oral PV in Thai patients. Early diagnosis and timely treatment are critical for the patients. Since oral mucosa is often the first site of PV involvement, healthcare professionals must recognize the disease's clinical characteristics and manage PV patients appropriately.

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Section: Discussionmentioning

confidence: 99%

Clinical profile of oral pemphigus vulgaris in Thai patients

Kaomongkolgit¹,

Sarideechaigul²,

Klanrit³

et al. 2021

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Section: Resultsmentioning

confidence: 99%

“…The studies were conducted in 20 countries: Argentina, 7 Brazil, 15,16 Croatia, 17 Egypt, 18 Greece, 19 India, 6,20–23 Iran, 24–29 Italy, 2,3,30 Japan, 31 Korea, 32 Kuwait, 33 Macedonia, 34 Mexico, 35 Morocco, 8,36 Romania, 37 Serbia and Montenegro, 38 Slovakia, 39 Spain, 40,41 Thailand, 42 and Turkey 1,5,9,43–45 . Years of publication ranged from 1999 33 to 2020 3,4,6–9,24 . The most common study design was observational study as retrospective reviews ( n = 20, 50%), followed by retrospective studies with follow‐up ( n = 18, 45%), and two prospective studies (5%).…”

Section: Resultsmentioning

confidence: 99%

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Prevalence of oral mucosal lesions in patients with pemphigus vulgaris: A systematic review and meta‐analysis

Batistella

Silva

Rivero

et al. 2021

J Oral Pathology Medicine

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Objective To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris. Methods Observational studies reporting the prevalence of oral lesions in pemphigus vulgaris patients, without restriction to language and year of publication, were selected in a two‐phase process. Search strategies were applied to PubMed, Scopus, Livivo, Web of Science, LILACS, Google Scholar, and OpenGrey databases. Articles assessing the prevalence of oral lesions in patients with conditions other than pemphigus vulgaris were excluded. Risk of bias analysis was performed using the Joanna Briggs Institute's Critical Appraisal Checklist for Studies Reporting Prevalence Data. Synthesis of results was calculated by the software R Statistics version 4.0.2 (The R Foundation). Confidence in cumulative evidence was assessed with the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) criteria. Results From 1957 studies identified, 40 were included in qualitative synthesis and 38 in meta‐analyses. The pooled prevalence of patients with oral lesions solely or concurrent with other mucocutaneous lesions was 90.3%. The prevalence of patients with exclusive oral mucosal lesions was 50.8%. Risk of bias was considered low, and the certainty of evidence was very low. Conclusion Oral lesions were present in approximately nine out of 10 patients with pemphigus vulgaris. The oral mucosa was the most common site of disease onset. Further longitudinal studies are urged to assess the prevalence of oral lesions at different disease stages.

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“…This clinical manifestation is due to the difference in timing of the presence of antibodies against Dsg 1 and Dsg 3. Several studies have shown that Dsg 3 antibodies were identified in 90–98% of all patients at the time of the initial diagnosis, while positive Dsg 1 antibodies were detected in only 63% of patients with PV at the time of diagnosis [ 13 ]. The clinical significance of this pathophysiological process in PV development explains the diagnostic delay: since around 60–80% of patients experience oral lesions first, and the disease remains limited to mucosal surfaces in 1 in 4 patients, diagnostic delays are commonly observed in PV [ 14 ].…”

Section: Clinical Presentationmentioning

confidence: 99%

Pemphigus—The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic

et al. 2021

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Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus. A diagnostic delay is very common in pemphigus, especially among patients with mucosal involvement. However, in recent years we have witnessed considerably fewer patients with extensive mucocutaneous manifestations, since patients with oral lesions are referred to dermatologists to start the treatment much sooner than they had been previously. Among non-classical variants of pemphigus, unusual cases with discrepancies between autoantibody profiles and clinics challenge the “desmoglein compensation theory”. The identification of several other autoantigens that perform a role in the pathogenesis of different variants of pemphigus will progress immunodermatology towards an approach that will determine personalized pemphigus subtypes for each patient. Comorbidities among patients are primarily associated with the prolonged use of corticosteroids and other immunosuppressive agents. The SARS-CoV-2 pandemic raised concerns regarding the immunosuppressive effects of treatment and the risk of a more complicated COVID-19 infection, as well as on the ability to develop an adequate vaccine response.

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Evaluation of desmoglein 1 and 3 autoantibodies in pemphigus vulgaris: correlation with disease severity

Cited by 12 publications

References 21 publications

Clinical profile of oral pemphigus vulgaris in Thai patients

Clinical profile of oral pemphigus vulgaris in Thai patients

Prevalence of oral mucosal lesions in patients with pemphigus vulgaris: A systematic review and meta‐analysis

Pemphigus—The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic

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