scholarly journals Immunization with desmoglein 3 induces non-pathogenic autoantibodies in mice

PLoS ONE ◽  
2021 ◽  
Vol 16 (11) ◽  
pp. e0259586
Author(s):  
Katharina Boch ◽  
Sören Dräger ◽  
Detlef Zillikens ◽  
Christoph Hudemann ◽  
Christoph M. Hammers ◽  
...  
Keyword(s):  
Disease Model ◽  
Pemphigus Vulgaris ◽  
Conformational Epitope ◽  
Autoimmune Blistering Disease ◽  
Immunization Strategies ◽  
Blistering Disease ◽  
Native Antigen ◽  
Circulating Autoantibodies ◽  
Monkey Esophagus

Background Pemphigus vulgaris (PV) is a rare autoimmune blistering disease characterized by the development of autoantibodies targeting desmoglein (Dsg) 3, but also against Dsg1 in mucocutaneous disease. Given that existing PV animal models only recapitulate aspects of the disease, we aimed to establish a more comprehensive disease model based on the immunization of mice with PV autoantigen(s). Methods The following immunization strategies were tested: (i) C57Bl/6J, B6.SJL-H2s C3c/1CyJ, DBA2/J, or SJL/J mice were immunized with recombinant murine Dsg3 (mDsg3), (ii) DBA2/J and SJL/J mice were immunized with mDsg3 and additionally injected a single non-blister inducing dose of exfoliative toxin A (ETA), and (iii) DBA2/J and SJL/J mice were immunized with human Dsg (hDsg) 1 and 3. Results Despite the induction of autoantibodies in each immunization protocol, the mice did not develop a clinical phenotype. Tissue-bound autoantibodies were not detected in the skin or mucosa. Circulating autoantibodies did not bind to the native antigen in indirect immunofluorescence microscopy using monkey esophagus as a substrate. Conclusion Immunization with PV autoantigens induced non-pathogenic Dsg1/3 antibodies, but did not cause skin/mucous membrane disease in mice. These findings, confirmed by failure of binding of the induced autoantibodies to their target in the skin, suggest that the autoantibodies which were formed were unable to bind to the conformational epitope present in vivo.

Sweet Syndrome and Pemphigus Vulgaris

2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Neutrophilic Dermatosis ◽  
Rare Presentation ◽  
Sweet Syndrome ◽  
Autoimmune Blistering Disease ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Erythematous Plaques ◽  
New Onset

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.

ICOS Gene Polymorphism May Be Associated with Pemphigus

2010 ◽  
Vol 14 (6) ◽  
pp. 291-297 ◽  
Author(s):  
Joanna Narbutt ◽  
Aleksandra Lesiak ◽  
Izabela Klich ◽  
Jolanta Dorota Torzecka ◽  
Anna Sysa-Jedrzejowska ◽  
...  
Keyword(s):  
Gene Polymorphism ◽  
Pemphigus Vulgaris ◽  
Control Group ◽  
Pemphigus Foliaceus ◽  
Autoimmune Blistering Disease ◽  
Genes Encoding ◽  
Blistering Disease ◽  
Ctla4 Gene ◽  
Genetically Determined ◽  
The Relationship

Background: Pemphigus is an autoimmune blistering disease mediated by circulating IgG autoantibodies directed against desmogleins 3 and/or 1. As pemphigus is a T cell–mediated disease, one may assume that genetically determined disregulation of costimulatory signal may be involved in its pathogenesis. Objective: The aim of the present study was to evaluate the relationship between polymorphisms in genes encoding costimulatory receptors, CTLA4 and ICOS, and pemphigus in the Polish population. Methods: The study included 54 patients with pemphigus: 40 with pemphigus vulgaris (PV) and 14 with pemphigus foliaceus (PF). Additionally, 176 healthy unrelated blood donors served as controls. +49A/G CTLA4 and IVS1+173 ICOS polymorphisms were identified using a modified polymerase chain reaction–restriction fragment-length polymorphism. Results: Analysis of the frequency of genotypes and alleles of +49A/G CTLA4 gene polymorphism showed no statistically significant differences between the PV and PF patients and the controls. The distribution of genotypes in IVS1+173 ICOS polymorphisms was significantly different in both PV ( p < .01) and PF ( p = .0004) patients when compared to controls. The carriers of the allele C were more frequent in PV or PF in comparison with the control group ( p < .001 for both groups). Conclusions: Our results suggest that genetically determined abnormal function of costimulatory receptors in T cells may be associated with the pathogenesis of pemphigus.

scholarly journals A Clinical Profile and Co-morbidities of Pemphigus Vulgaris Patients: A Study of 35 Cases

KYAMC Journal ◽  
2019 ◽  
Vol 10 (2) ◽  
pp. 106-109
Author(s):  
Md Abdur Razzaque ◽  
Zulfikar Ali ◽  
Kazi Shihab Uddin ◽  
Md Imtiajul Islam ◽  
Sajib Kumar Nath
Keyword(s):  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Cutaneous Lesions ◽  
Female Ratio ◽  
Medical College ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease ◽  
High Index Of Suspicion ◽  
Male To Female ◽  
Sixth Decade

Background: Pemphigus vulgaris (PV), an autoimmune blistering disease involving the skin and mucosa. PV frequently begins with oral lesions and progresses to skin lesions. Autoimmune bullous skin disorders are associated with IgG or IgA auto- antibodies against distinct adhesion molecules of the epidermis and dermal epidermal basement membrane zone, respectively. These auto- antibodies lead to a loss of skin adhesion which shows up clinically as the formation of blisters or erosions. Objectives: To characterize the clinical parameters and co-morbidities of PV patients from a single tertiary medical centre in Bangladesh. Material and Methods: This observational study was done including 35 PV patients attending in the department of Dermatology and Venereology, Khwaja Yunus Ali Medical College Khawja Eunus Ali Medical College from 2010 to 2014. Thirty patients of pemphigus diagnosed clinically confirmed and treated over a 4-year period (2010-2014). Results: Majority of the patients 45.7% belongs to age group 41-50 years. Mean age 47.12±11.13. The male to female ratio in our study sample was 1:1.5. The youngest patient was 17 years old and the oldest 68. For both genders, the risk of onset peaked during the fifth and sixth decade of life. Out of 35 patients, 15(42.9%) was presented with mucosal lesions only, while 13 patients 37.1% had mucocutaneous lesions and 7 patients 20% had only cutaneous lesions. The most common comorbidies were hypertension 20.0%, osteoporosis 17.1%, and diabetes 8.6%, thyroid disease 8.6%, psoriasis 5.7%, rheumatoid arthritis 2.7%, rheumatic fever 2.9%, autoimmune hepatitis 2.9%, and myasthenia gravis 2.9%. Conclusion: The associated comorbidities of PV emphasize the need for dermatologists to keep a high index of suspicion and actively evaluate patients to determine their presence. KYAMC Journal Vol. 10, No.-2, July 2019, Page 106-109

scholarly journals Pemphigus Vulgaris Presented with Cheilitis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Zaheer Abbas ◽  
Zahra Safaie Naraghi ◽  
Elham Behrangi
Keyword(s):  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Lower Lip ◽  
Additional Lesion ◽  
Autoimmune Blistering Disease ◽  
Systemic Corticosteroids ◽  
Erosive Lichen Planus ◽  
Blistering Disease ◽  
One Year

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. But it is unusual for the disease to present with initial and solitary persistent lower lip lesions without progression to any other location.Main Observations. We report a 41-year-old woman with dry crusted lesions only on the lower lip, clinically resembling actinic cheilitis and erosive lichen planus, but histopathological evaluation showed unexpected results of suprabasal acantholysis and cleft compatible with pemphigus vulgaris. We treated her with intralesional triamcinolone 10 mg/mL for 2 sessions and 2 g cellcept daily. Patient showed excellent response and lesions resolved completely within 2 months. In one-year follow-up, there was no evidence of relapse or any additional lesion on the other sites.Conclusion. Cheilitis may be the initial and sole manifestation of pemphigus vulgaris. Localized and solitary lesions of pemphigus vulgaris can be treated and controlled without systemic corticosteroids.

scholarly journals Anti-p200 pemphigoid: A review

2021 ◽  
Vol 0 ◽  
pp. 1-6
Author(s):  
Vattiyamveetil Sajeela Rasheed
Keyword(s):  
Clinical Features ◽  
Autoimmune Blistering Disease ◽  
Mucosal Involvement ◽  
Blistering Disease ◽  
Circulating Autoantibodies

Anti-p200 pemphigoid, initially described in 1996, is a subepidermal autoimmune blistering disease. It is manifested as tense blisters, mostly in an acral distribution, and is accompanied by mucosal involvement in more than half the cases. The disease is produced by circulating autoantibodies directed against the dermal antigen of 200-kDa, the exact identity of which remains unknown. This review focuses on the clinical features, immunopathogenesis, and diagnosis of anti-p200 pemphigoid.

Mean Platelet Volume in Pemphigus Vulgaris

Angiology ◽  
2017 ◽  
Vol 69 (4) ◽  
pp. 303-307 ◽  
Author(s):  
Khalaf Kridin ◽  
Wesal Shihade ◽  
Shira Zelber-Sagi
Keyword(s):  
Mean Platelet Volume ◽  
Pemphigus Vulgaris ◽  
Platelet Volume ◽  
Inflammatory Parameters ◽  
Autoimmune Blistering Disease ◽  
Before And After ◽  
Blistering Disease ◽  
The Mean ◽  
Study Participants ◽  
Control Study

Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes. The mean platelet volume (MPV) is an index of platelet activation reported to be influenced by inflammation. We investigated the MPV in patients with PV relative to controls. This case–control study included 160 patients with PV and 640 age- and sex-matched controls. The MPV, platelet count, and other inflammatory parameters were measured for all study participants. A significantly decreased MPV was observed in patients with PV compared with controls, 9.1 (1.5) fL versus 10.3 (1.4) fL, P < .001. A significant negative association between MPV and PV was demonstrated in multivariate analysis (odds ratio, 0.59; 95% confidence interval, 0.51-0.68, P < .0001). Patients having PV with laryngeal involvement had significantly lower MPVs than those without involvement of the larynx, 8.81 (1.5) fL versus 9.32 (1.4) fL, P = .032. Disease activity (before and after treatment) does not affect the MPV. Patients with PV showed decreased MPV values compared to healthy controls. The MPV may be a feasible diagnostic marker in patients with PV.

scholarly journals Epidemiology and Factors Associated with Remission of Pemphigus Vulgaris and Foliaceus in Singapore

2020 ◽  
Vol 49 (6) ◽  
pp. 367-376
Author(s):  
Sophie CS Cai ◽  
Xiahong Zhao ◽  
Mark BY Tang ◽  
Yen Loo Lim
Keyword(s):  
Antibody Titre ◽  
Pemphigus Vulgaris ◽  
National Registry ◽  
Pemphigus Foliaceus ◽  
Prednisolone Dose ◽  
Asian Patients ◽  
Autoimmune Blistering Disease ◽  
Antibody Titres ◽  
Blistering Disease ◽  
Study Methodology

Background: Pemphigus is a chronic, relapsing immunobullous disease. There is limited data on the clinical course and prognostic factors of pemphigus in Asian patients. Methods: We conducted a retrospective cohort study of all newly diagnosed pemphigus vulgaris (PV) and pemphigus foliaceus (PF) patients seen at the National Skin Centre from 1 January 2004 to 31 December 2009. Demographic and clinical data on co-morbidities, treatment and remission were recorded. Mortality information was obtained from the National Registry of Diseases. Prognostic endpoint was overall remission at last visit. Results: Sixty- one patients (36 PV and 25 PF) were recruited. Among PV patients, higher initial prednisolone dose (P = 0.017) and the use of azathioprine (P = 0.028) were significantly associated with overall remission at last visit. However, higher desmoglein 1 antibody titres at diagnosis (P = 0.024) and the use of dapsone (P = 0.008) were negatively associated with overall remission at last visit. Among PF patients, only higher desmoglein 1 antibody titre at diagnosis (P = 0.041) was found to be associated with lower overall remission at last visit. There was no mortality during the 3-year follow-up period in both PV and PF. Conclusions: Higher initial prednisolone dose and the use of azathioprine in PV desmoglein 1 antibody titre at diagnosis in PV and PF might be prognostic markers for achieving remission. Use of dapsone was associated with lower overall remission in PV, but this might be confounded because dapsone was used as an adjuvant therapy in recalcitrant cases. Owing to study methodology and limitations, further evaluation is needed for better prognostication of pemphigus. Ann Acad Med Singapore 2020;49:367–76 Key words: Autoimmune blistering disease, Dermatology, Pemphigus foliaceus, Pemphigus vulgaris, Remission

scholarly journals Acometimento neonatal do pênfigo vulgar: relato de caso

2020 ◽  
Vol 9 (2) ◽  
Author(s):  
Mariana das Neves Melo ◽  
Elisangela Pegas
Keyword(s):  
Risk Factors ◽  
Case Report ◽  
Fungal Infections ◽  
Pemphigus Vulgaris ◽  
Review Of Literature ◽  
Pregnancy Risk ◽  
Vertical Transfer ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease ◽  
In Pregnancy

Os pênfigos são dermatoses bolhosas autoimunes, em que há a produção de autoanticorpos direcionados contra moléculas de adesão dos epitélios, levando à perda da coesão celular. As doenças bolhosas intraepidérmicas são compostas pelo grupo dos pênfigos: pênfigo foliáceo clássico, pênfigo foliáceo endêmico, pênfigo eritematoso, pênfigo vulgar, pênfigo vegetante, pênfigo herpetiforme (variante clínica dos pênfigos vulgar e foliáceo), pênfigo por IgA, pênfigo paraneoplásico e pênfigo induzido por medicamentos. Devido à transferência placentária dos anticorpos IgG, existem casos de recém-nascidos com diagnóstico de Pênfigo Neonatal. Este  artigo relata o caso de uma paciente recém-nascida de mãe com diagnóstico prévio de Pênfigo Vulgar com lesões exulceradas extensas na região perineal, couro cabeludo, cervical posterior e dorso, e também lesões bolhosas nos pés.Descritores: Pênfigo; Recém-Nascido; Dermatopatias; Doenças Autoimunes.ReferênciasSmolinski KN, Shah SS, Honig PJ, Yan AC. Neonatal cutaneous fungal infections. Curr Opin Pediatr. 2005;17(4):486-93.Zhao CY, Chiang YZ, Murrell DF. Neonatal autoimmune blistering disease: a systematic review. Pediatr Dermatol. 2016;33(4):367-74.Bellelli AG, Mantero NM, Rueda ML, Navacchia D, Cao G, Liloo L et al. Pénfigo vulgar de la infancia, a propósito de un caso [Childhood pemphigus vulgaris, a case report]. Arch Argent Pediatr. 2016;114(6):e457-60. Ali HS. Pemphigus vulgaris during pregnancy - a case report. J Pak Assoc Dermatol. 2011;21(4):301-3.Hanna S, Kim M, Murrell DF. Validation studies of outcome measures in pemphigus. Int J Womens Dermatol. 2016;2(4):128-39.Ibrahim SB, Yashodhara BM, Umakanth S, Kanagasabai S. Pemphigus vulgaris in a pregnant woman and her neonate. BMJ Case Rep. 2012;2012:bcr0220125850.Kodagali SS, Subbarao SD, Hiremagaloor R. Pemphigus vulgaris in a neonate and his mother. Indian Pediatr. 2014;51(4):316-17.Fainaru O, Mashiach R, Kupferminc M, Shenhav M, Pauzner D, Lessing JB. Pemphigus vulgaris in pregnancy: a case report and review of literature. Hum Reprod. 2000;15(5):1195-97.Abrams ML, Smidt A, Benjamin L, Chen M, Woodley D, Mancini AJ. Congenital epidermolysis bullosa acquisita: vertical transfer of maternal autoantibody from mother to infant. Arch Dermatol. 2011;147(3):337-41.Goldberg NS, DeFeo C, Kirshenbaum N. Pemphigus vulgaris and pregnancy: risk factors and recommendations. J Am Acad Dermatol. 1993;28(5 Pt 2):877-79.

scholarly journals Radiation-induced pemphigus in a patient with an invasive ductal carcinoma of the breast: a case report

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Charles Crocker
Keyword(s):  
Ductal Carcinoma ◽  
Pemphigus Vulgaris ◽  
External Beam Radiation Therapy ◽  
External Beam Radiation ◽  
Carcinoma Of The Breast ◽  
Beam Radiation ◽  
Autoimmune Blistering Disease ◽  
History Of ◽  
Blistering Disease ◽  
Radiation Induced

Abstract A 72-year-old woman with a known history of breast cancer was treated with adjuvant external beam radiation therapy. She initially developed radiation field localized blistering and erosions of the skin, before developing more widespread lesions. Immunofluorescence confirmed pemphigus vulgaris, which was responsive to corticosteroids. Pemphigus vulgaris is an autoimmune blistering disease of the skin that can rarely be associated with exposure to ionizing radiation.

scholarly journals A simple epithelial cell line (MDCK) shows heterogeneity of desmoglein isoforms, one resembling pemphigus vulgaris antigen

1995 ◽  
Vol 108 (4) ◽  
pp. 1743-1750
Author(s):  
M.J. Vilela ◽  
T. Hashimoto ◽  
T. Nishikawa ◽  
A.J. North ◽  
D. Garrod
Keyword(s):  
Epithelial Cell ◽  
Cell Line ◽  
Mdck Cells ◽  
Pemphigus Vulgaris ◽  
Epithelial Cell Line ◽  
Cell Type ◽  
Epithelial Cell Type ◽  
Blistering Disease ◽  
Circulating Autoantibodies ◽  
Canine Kidney

The epidermal blistering disease, pemphigus vulgaris (PV), is caused by circulating autoantibodies that react with a desmosomal glycoprotein desmoglein (Dsg3). This antigen is expressed only in stratified epithelial tissues. Here we show that the simple epithelial canine kidney cell line, MDCK, expresses at least two desmoglein isoforms recognised by different monoclonal antibodies. One of these isoforms is a 130 × 10(3) M(r) polypeptide that is recognised by both PV autoantisera and a monoclonal antibody reactive with a cytoplasmic domain of human Dsg3. Antibodies in PV sera bind to the surface of MDCK cells but not cause loss of intercellular adhesion. This is the first demonstration of the expression of a polypeptide related to human PV antigen by a simple epithelial cell type.

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