Abstract
Autoimmune bullous skin disorders represent a group of severe, chronic skin diseases which are characterized by the presence of autoantibodies targeting distinct adhesionmolecules of the epidermis and dermoepidermal basement membrane zone leading to a loss of adhesive function of the target antigen(s) (Fig. 8.1). The appearance of blisters and erosions of the skin and/or mucous membranes is the leading clinical sign of autoimmune bullous skin disorders (Fig. 8.2). While histopathology reveals the location of the blister formation, the detection of tissue bound autoantibodies by immunofluorescent staining of uninvolved perilesional skin biopsies is mandatory for diagnosing autoimmune bullous skin disorders. Circulating autoantibodies can be visualized by indirect immunofluorescence using tissue substrates such as monkey esophagus and sodium chloride-split human skin. Based on the specificity of the targeted antigens, several clinically and immune serologically distinct bullous disorders have been defined (Fig. 8.2).
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