Bullous disorders

Author(s):  
Emily Davies

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.

1996 ◽  
Vol 106 (6) ◽  
pp. 1277-1280 ◽  
Author(s):  
John J. Zone ◽  
Ted B. Taylor ◽  
Donald P. Kadunce ◽  
Tadeusz P. Chorzelski ◽  
Lawrence A. Schachner ◽  
...  

2012 ◽  
Vol 3 (1) ◽  
pp. 68-73
Author(s):  
BR Premalatha ◽  
Roopa S Rao ◽  
Vijaya Mysorekar

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.


2010 ◽  
pp. 4602-4609
Author(s):  
Fenella Wojnarowska

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....


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