scholarly journals Bullous Skin Diseases: Classical Types of Autoimmune Diseases

Scientifica ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Jan Damoiseaux
Keyword(s):  
Autoimmune Diseases ◽  
Bullous Pemphigoid ◽  
Oral Mucosa ◽  
Skin Diseases ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Blister Formation ◽  
Koch’S Postulates ◽  
Bullous Skin Diseases ◽  
Koch's Postulates

The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemi)desmosomes. Koch’s postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch’s postulates are fulfilled, and, therefore, these bullous skin diseases are to be considered classical autoimmune diseases within the wide and expanding spectrum of autoimmune diseases.

The Comparative Pathology of Non-Viral Bullous Skin Diseases in Domestic Animals

1980 ◽  
Vol 17 (3) ◽  
pp. 257-281 ◽  
Author(s):  
D. W. Scott ◽  
M. J. Wolfe ◽  
C. A. Smith ◽  
R. M. Lewis
Keyword(s):  
New York ◽  
Epidermolysis Bullosa ◽  
Lupus Erythematosus ◽  
Skin Diseases ◽  
New York State ◽  
Pemphigus Vulgaris ◽  
Domestic Animals ◽  
Pemphigus Foliaceus ◽  
Epidermolysis Bullosa Simplex ◽  
Bullous Skin Diseases

In a review of non-viral bullous skin diseases of domestic animals and a 4-year study of cases presented to the New York State College of Veterinary Medicine, we found 15 diseases: pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, bullous pemphigoid, systemic lupus erythematosus, dermatitis herpetiformis, toxic epidermal necrolysis, drug eruption, epidermolysis bullosa, epidermolysis bullosa simplex, familial acantholysis, bovine congenital porphyria, impetigo and subcorneal pustular dermatosis. The 15 diseases were placed in five categories: autoimmune, immune-mediated, hereditary, bacterial and idiopathic. A histologic classification of these disorders based on the site of blister formation and other important clinicopathologic, histologic and immunopathologic findings was developed.

AI-Provided Instant Differential Diagnosis of Pemphigus Vulgaris and Bullous Pemphigoid: Qualitative Study (Preprint)

2020 ◽  
Author(s):  
Xiaoyu He ◽  
Juan Su ◽  
Guangyu Wang ◽  
Kang Zhang ◽  
Navarini Alexander ◽  
...  
Keyword(s):  
Artificial Intelligence ◽  
Early Diagnosis ◽  
Image Classification ◽  
Bullous Pemphigoid ◽  
Skin Diseases ◽  
Pemphigus Vulgaris ◽  
Classification Model ◽  
Clinical Image ◽  
Clinical Images ◽  
Multimodal Classification

BACKGROUND Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are two rare but severe inflammatory dermatoses. Due to the regional lack of trained dermatologists, many patients with these two diseases are misdiagnosed and therefore incorrectly treated. An artificial intelligence diagnosis framework would be highly adaptable for the early diagnosis of these two diseases. OBJECTIVE Design and evaluate an artificial intelligence diagnosis framework for PV and BP. METHODS The work was conducted on a dermatological dataset consisting of 17,735 clinical images and 346 patient metadata of bullous dermatoses. A two-stage diagnosis framework was designed, where the first stage trained a clinical image classification model to classify bullous dermatoses from five common skin diseases and normal skin and the second stage developed a multimodal classification model of clinical images and patient metadata to further differentiate PV and BP. RESULTS The clinical image classification model and the multimodal classification model achieved an area under the receiver operating characteristic curve (AUROC) of 0.998 and 0.942, respectively. On the independent test set of 20 PV and 20 BP cases, our multimodal classification model (sensitivity: 0.85, specificity: 0.95) performed better than the average of 27 junior dermatologists (sensitivity: 0.68, specificity: 0.78) and comparable to the average of 69 senior dermatologists (sensitivity: 0.80, specificity: 0.87). CONCLUSIONS Our diagnosis framework based on clinical images and patient metadata achieved expert-level identification of PV and BP, and is potential to be an effective tool for dermatologists in remote areas in the early diagnosis of these two diseases.

Viruses and autoimmune diseases—adapting Koch's postulates

2004 ◽  
Vol 3 (5) ◽  
pp. 355-361 ◽  
Author(s):  
A.M Denman ◽  
Bracha Rager-Zisman
Keyword(s):  
Autoimmune Diseases ◽  
Koch’S Postulates ◽  
Koch's Postulates

scholarly journals Inhibition of Rho A activity causes pemphigus skin blistering

2006 ◽  
Vol 175 (5) ◽  
pp. 721-727 ◽  
Author(s):  
Jens Waschke ◽  
Volker Spindler ◽  
Paola Bruggeman ◽  
Detlef Zillikens ◽  
Gudula Schmidt ◽  
...  
Keyword(s):  
Skin Diseases ◽  
Pemphigus Vulgaris ◽  
Mitogen Activated Protein Kinase ◽  
Pemphigus Foliaceus ◽  
Laser Tweezers ◽  
Desmosomal Cadherins ◽  
Skin Cell ◽  
Mitogen Activated Protein ◽  
Rho A

The autoimmune blistering skin diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are mainly caused by autoantibodies against desmosomal cadherins. In this study, we provide evidence that PV–immunoglobulin G (IgG) and PF-IgG induce skin blistering by interference with Rho A signaling. In vitro, pemphigus IgG caused typical hallmarks of pemphigus pathogenesis such as epidermal blistering in human skin, cell dissociation, and loss of desmoglein 1 (Dsg 1)–mediated binding probed by laser tweezers. These changes were accompanied by interference with Rho A activation and reduction of Rho A activity. Pemphigus IgG–triggered keratinocyte dissociation and Rho A inactivation were p38 mitogen-activated protein kinase dependent. Specific activation of Rho A by cytotoxic necrotizing factor-y abolished all pemphigus-triggered effects, including keratin retraction and release of Dsg 3 from the cytoskeleton. These data demonstrate that Rho A is involved in the regulation of desmosomal adhesion, at least in part by maintaining the cytoskeletal anchorage of desmosomal proteins. This may open the possibility of pemphigus treatment with the epidermal application of Rho A agonists.

scholarly journals Contemporary aspects on the immunopathogenesis of pemphigus group in the dog

2017 ◽  
Vol 55 (4) ◽  
pp. 319
Author(s):  
E. M. PAPADOGIANNAKIS (E. Ι. ΠΑΠΑΔΟΓΙΑΝΝΑΚΗΣ)
Keyword(s):  
Immune System ◽  
Autoimmune Diseases ◽  
Adhesion Molecules ◽  
Successful Treatment ◽  
Skin Diseases ◽  
Therapeutic Strategies ◽  
Review Article ◽  
Molecular Techniques ◽  
Pemphigus Foliaceus ◽  
Recent Advances

Autoimmune diseases of the canine epidermis originate from the activation of the immune system against some adhesion molecules, which link the keratinocytes. Epidermal autoimmunity eventually induces acantholysis, which is thepathological hallmark of these skin diseases. In this review article, a thorough analysis of the immunopathogenesis for each of these skin diseases has been attempted along with the mechanisms that modulate the process of acantholysis. Epidermal autoimmune diseases in the dog include pemphigus foliaceus, vulgaris, panepidermal pustular, erythematosus, paraneoplastic and pharmaceutical. The recent advances in immunopathological and molecular techniques have markedly facilitated the understanding of their pathogenesis, thus giving the opportunity for the development of new therapeutic strategies that may lead to their successful treatment.

scholarly journals Utility of oral mucosa as a substrate for the serodiagnosis of pemphigus: A descriptive analysis

2021 ◽  
Vol 0 ◽  
pp. 1-6
Author(s):  
Anuradha Jindal ◽  
Chythra Rao ◽  
Satish B. Pai ◽  
Raghavendra Rao
Keyword(s):  
Human Skin ◽  
Oral Mucosa ◽  
Immunosorbent Assay ◽  
Indirect Immunofluorescence ◽  
Pemphigus Vulgaris ◽  
Enzyme Linked Immunosorbent Assay ◽  
Pemphigus Foliaceus ◽  
Normal Human Skin ◽  
Normal Human ◽  
Monkey Esophagus

Background: The indirect immunofluorescence test is useful in the serodiagnosis of pemphigus. As indirect immunofluorescence titers correlate with disease activity in pemphigus, it is often used as a monitoring tool. The sensitivity of indirect immunofluorescence depends on the substrate used, and the preferred substrates are monkey esophagus for pemphigus vulgaris and normal human skin for pemphigus foliaceus. Aims: We evaluated oral mucosa as a substrate for indirect immunofluorescence in pemphigus. Methods: Fifty patients with pemphigus (40 with pemphigus vulgaris and ten with pemphigus foliaceus) and 50 controls were enrolled for study. Demographic and clinical details were recorded and indirect immunofluorescence using two substrates (oral mucosa and normal human skin) was carried out in serial dilution. Desmoglein (Dsg) 1 and 3 enzyme-linked immunosorbent assay was also evaluated simultaneously. Results: Indirect immunofluorescence was positive in 40 patients (80%) with oral mucosa substrate and 34 patients (68%) with normal human skin substrate. Circulating antibodies were detected with oral mucosa in 33 (82.5%) of the 40 pemphigus vulgaris patients and in 26 (65%) patients using normal human skin. Antibodies were detected in eight of the ten pemphigus foliaceus patients (80%) with normal human skin and in seven (70%) patients with oral mucosa. Dsg enzyme-linked immunosorbent assay was positive in 45 (90%) patients, and 37 of these were also indirect immunofluorescence positive with oral mucosa. In the five Dsg enzyme-linked immunosorbent assay-negative patients, indirect immunofluorescence with oral mucosa was positive in three. Limitations: A comparison of oral mucosa with monkey esophagus could not be performed. Conclusion: Oral mucosa is a suitable and sensitive substrate for indirect immunofluorescence in pemphigus. Further studies comparing the sensitivity of indirect immunofluorescence using oral mucosa with monkey esophagus are recommended.

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