Vesiculobullous disease

2010 ◽  
pp. 4602-4609
Author(s):  
Fenella Wojnarowska
Keyword(s):  
Basement Membrane ◽  
Clinical Presentation ◽  
Dermatitis Herpetiformis ◽  
Basement Membrane Zone ◽  
Genetic Mutations ◽  
Bullous Diseases ◽  
Pathogenic Antibodies ◽  
Adhesion Complex ◽  
Substantial Morbidity ◽  
Autoimmune Blistering Diseases

The autoimmune blistering diseases have a dramatic clinical presentation, and are significant diseases with substantial morbidity and mortality. The diseases can be split broadly pathologically into intraepidermal (pemphigus) and subepidermal (pemphigoid, dermatitis herpetiformis and others) groups, the former being characterized by pathogenic autoantibodies to desmosome components, and the latter by pathogenic antibodies to proteins of the basement membrane zone adhesion complex that link the epithelium/epidermis to the underlying mesenchyme/dermis (or genetic mutations of the same proteins). There are concomitant differences in clinical presentation, e.g. blistering lesions present in the subepidermal bullous diseases tend to be less easily ruptured than those observed in intraepidermal bullous diseases....

Bullous disorders

2018 ◽  
Author(s):  
Emily Davies
Keyword(s):  
Basement Membrane ◽  
Dermatitis Herpetiformis ◽  
Pemphigus Vulgaris ◽  
Basement Membrane Zone ◽  
Mucous Membrane Pemphigoid ◽  
Pemphigus Foliaceus ◽  
Pemphigoid Gestationis ◽  
Bullous Disease ◽  
Adhesion Junctions ◽  
Linear Iga Disease

This chapter focuses on immunobullous diseases. The immunobullous disorders are a group of diseases in which pathogenic autoantibodies bind to target antigens either in desmosomes (intra-epidermal intracellular adhesion junctions) or in part of the basement membrane zone, resulting in loss of adhesion, and blister formation. This chapter will focus on pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, linear IgA disease, chronic bullous disease of childhood, and dermatitis herpetiformis; it will also mention mucous membrane pemphigoid, pemphigoid gestationis, and epidermolysis bullosa acquisita.

scholarly journals Immunofluorescence in Oral Pathology—Part II: Pathology and Immunofluorescent Patterns in Subepidermal Immunobullous Disorders

2012 ◽  
Vol 3 (1) ◽  
pp. 68-73
Author(s):  
BR Premalatha ◽  
Roopa S Rao ◽  
Vijaya Mysorekar
Keyword(s):  
Basement Membrane ◽  
Skin Biopsy ◽  
Oral Pathology ◽  
Basement Membrane Zone ◽  
Cicatricial Pemphigoid ◽  
Clinicopathological Correlation ◽  
Complement Components ◽  
Bullous Disease ◽  
Exhaustive List ◽  
Autoimmune Blistering Diseases

ABSTRACT The immunobullous disorders are a group of autoimmune diseases in which components of the epidermis and basement membrane zone are targeted, resulting in the formation of cutaneous and mucosal blisters. Based on the level of blistering, the autoimmune blistering diseases may be subdivided into intraepidermal and subepidermal. An exhaustive list of immunobullous disorders is beyond the scope of this review, but those involving oral mucosa are taken into consideration. One major group namely the subepidermal immunobullous diseases which includes bullous pemphigoid (BP), mucosal pemphigoid [cicatricial pemphigoid (CP) or (MMP)], epidermolysis bullosa acquisita (EBA) linear IgA bullous disease (LABD) are discussed in this section. The diagnosis of these diseases requires clinicopathological correlation; immunofluorescence methods provide a useful adjunct to light microscopy. These methods entail the use of fluorescein-linked antibodies to immunoglobulins, complement components, or other proteins either in the skin biopsy or sera. In continuation with part I, the immunofluorescence patterns in the above listed immunobullous disorders are reviewed in detail with a summary of pathogenesis and characteristic histopathological findings. How to cite this article Rao RS, Premalatha BR, Mysorekar V. Immunofluorescence in Oral Pathology—Part II: Pathology and Immunofluorescent Patterns in Subepidermal Immunobullous Disorders. World J Dent 2012;3(1):68-73.

Analysis of wound healing in an in vitro model: early appearance of laminin and a 125 × 10(3) Mr polypeptide during adhesion complex formation

1990 ◽  
Vol 96 (4) ◽  
pp. 651-660
Author(s):  
M.A. Kurpakus ◽  
E.L. Stock ◽  
J.C. Jones
Keyword(s):  
Wound Healing ◽  
Basement Membrane ◽  
In Vitro Model ◽  
Collagen Type ◽  
Basement Membrane Zone ◽  
Lamina Densa ◽  
Vitro Model ◽  
Collagen Type Vii ◽  
Adhesion Complex

The adhesion complex, which plays an important role in cell-substratum attachment, consists of a cellular hemidesmosomal plaque, anchoring filaments, the basement membrane zone and anchoring fibrils. An analysis of the temporal sequence of assembly of the adhesion complex was undertaken in an in vitro model of epithelial cell wound healing by immunofluorescence and electron microscopy. A monoclonal antibody directed against a 125K (K = 10(3) Mr) polypeptide (mAbHD), bullous pemphigoid (BP) autoantibodies, antibodies directed against collagen type VII and laminin antibodies were used as markers for anchoring filaments, the hemidesmosome, anchoring fibrils and the laminin component of the basement membrane zone, respectively. Fluorescence labeling could be detected with mAbHD before labeling with BP autoantibodies or collagen type VII antibodies. Laminin fluorescence was detected at the same time as mAbHD. Furthermore, the 125K polypeptide and laminin were located extracellularly prior to the appearance of BP antigen and collagen type VII. The appearance of the hemidesmosomal plaque at the electron microscope level succeeded the localization of BP antigen in basal cells detected by immunofluorescence microscopy. No evidence for the coordinated appearance of BP antigen, collagen type VII and laminin was observed in this model. We discuss the possibility that the 125K protein and laminin may play roles in the initiation of complex formation. Furthermore, although basement membrane zone components were detected early in adhesion complex re-formation, formation of the lamina densa region of the basement membrane zone followed the appearance of the hemidesmosomal plaque, indicating a role for the hemidesmosomal plaque in organizing the structure of the lamina densa.

scholarly journals Detection of anti-basement membrane zone antibodies in the blister fluid in subepidermal autoimmune bullous diseases

2017 ◽  
Vol 62 (6) ◽  
pp. 541
Author(s):  
Raghavendra Rao ◽  
Ravindran Surya ◽  
Bobbili Tejasvi ◽  
ShrutakirthiD Shenoi ◽  
Sathish Pai ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Basement Membrane Zone ◽  
Blister Fluid ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases

Autoimmune bullous diseases

2020 ◽  
pp. 5612-5620
Author(s):  
Kathy Taghipour ◽  
Fenella Wojnarowska
Keyword(s):  
Basement Membrane ◽  
Basal Cell ◽  
Epidermolysis Bullosa ◽  
Cell Layer ◽  
Basement Membrane Zone ◽  
Epidermolysis Bullosa Acquisita ◽  
Significant Morbidity ◽  
Heterogenous Group ◽  
Autoimmune Bullous Diseases ◽  
Bullous Diseases

Autoimmune bullous diseases of the skin are a heterogenous group of blistering diseases that affect the skin and/or mucosal membranes. They are associated with significant morbidity and mortality and may present to several different specialists. They are broadly divided into two groups depending on the location of the blisters formed in the skin, which may be subepidermal (pemphigoids, linear IgA disease, dermatitis herpetiformis, epidermolysis bullosa acquisita) or intraepidermal (pemphigus group). Pathogenic autoantibodies (IgG, IgA) target either the proteins that provide keratinocyte adhesion (intraepidermal disease) or the proteins of hemidesmosomes that attach the basal cell layer to the basement membrane zone (subepidermal disease).

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