scholarly journals Immunoblot Analysis Using Recombinant Protein of the 180kD Bullous Pemphigoid Antigen NC16a Domain as an Aid to the Diagnosis of Atypical Subepidermal Autoimmune Bullous Skin Diseases.

1995 ◽  
Vol 44 (2) ◽  
pp. 62-66 ◽  
Author(s):  
Takashi Hashimoto ◽  
Kunie Matsumura ◽  
Yoshie Kawahara ◽  
Yoshiyuki Ohata ◽  
Takeji Nishikawa
Keyword(s):  
Recombinant Protein ◽  
Bullous Pemphigoid ◽  
Skin Diseases ◽  
Immunoblot Analysis ◽  
Bullous Pemphigoid Antigen ◽  
Bullous Skin Diseases

Autoimmune bullous disorders

2006 ◽  
Vol 44 (2) ◽  
Author(s):  
Rüdiger Eming ◽  
Michael Hertl
Keyword(s):  
Basement Membrane ◽  
Skin Diseases ◽  
Immunoblot Analysis ◽  
Primary Diagnosis ◽  
Basement Membrane Zone ◽  
Epidermal Keratinocytes ◽  
Direct Immunofluorescence ◽  
Organ Specific ◽  
Circulating Autoantibodies ◽  
Bullous Skin Diseases

AbstractBullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the epidermis and the dermo-epidermal basement membrane zone. Binding of these autoantibodies to their antigenic targets results in loss of adhesion between epidermal keratinocytes and at the level of the basement membrane zone. Chronic blisters and secondary painful erosions are the clinical hallmark of autoimmune bullous disorders. Histopathology reveals the location of blister formation and helps to classify the subtype of the bullous skin disorder. Immunofluorescence is crucial for diagnosing autoimmune bullous skin disorders. Tissue-bound autoantibodies are detected by direct immunofluorescence of perilesional skin. Circulating autoantibodies can be visualised by indirect immunofluorescence using tissue substrates such as monkey oesophagus and sodium chloride-split human skin. Most of the autoantigens are available as recombinant proteins, which allows for autoantibody screening by ELISA or immunoblot analysis to confirm the primary diagnosis and, importantly, for immunoserological follow-up of patients.

scholarly journals Degradation of the epidermal-dermal junction by proteolytic enzymes from human skin and human polymorphonuclear leukocytes.

1984 ◽  
Vol 160 (4) ◽  
pp. 1027-1042 ◽  
Author(s):  
R A Briggaman ◽  
N M Schechter ◽  
J Fraki ◽  
G S Lazarus
Keyword(s):  
Human Skin ◽  
Bullous Pemphigoid ◽  
Polymorphonuclear Leukocytes ◽  
Skin Diseases ◽  
Proteolytic Enzymes ◽  
Cathepsin G ◽  
Basal Cells ◽  
Lamina Densa ◽  
Normal Human Skin ◽  
Bullous Pemphigoid Antigen

The degradation of normal human skin by the human polymorphonuclear leukocyte proteinases cathepsin G and elastase, and by a human skin chymotrypsin-like proteinase that appears to be a mast cell constituent, was examined. Enzymes were incubated with fresh, split-thickness skin for up to 8 h; the tissue was examined ultrastructurally and immunohistochemically using antibodies to known basement membrane constituents. In all cases, the primary damage observed was at the epidermal-dermal junction. Elastase degraded the lamina densa leaving scattered and disorganized anchoring fibrils, dermal microfibril bundles, and normal-appearing collagen fibers. Immunohistochemically, type IV collagen, laminin, KF1 antigen, and EBA antigen were absent. The bullous pemphigoid antigen was present and localized on the basal cells. Epidermal-dermal separation produced by the chymotrypsin-like proteinases, cathepsin G, and the human skin proteinase, was confined to the lamina lucida. The lamina densa and sub-lamina densa fibrillar network remained intact. The human skin chymotrypsin-like proteinase produced extensive epidermal-dermal separation, while cathepsin G, at comparable concentrations, produced only focal separations. Immunohistochemically, all antigens were present after incubation with enzyme. The bullous pemphigoid antigen, however, was found on the epidermal side of the split, while laminin was found on the dermal side. These results show that the epidermal-dermal junction is highly susceptible to neutral serine proteinases located in mast cells and polymorphonuclear leukocytes. Although all the proteinases produce epidermal-dermal separation, the patterns and extent of degradation are different. The distinctive patterns of degradation may provide a clue to the involvement of these proteinases in skin diseases.

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.

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.

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