Autoimmune blistering disorders

Linear IgA bullous disease is an autoimmune subepider-mal disorder, characterized by a linear deposition of IgA along the blister base, with a predominantly neutrophilic dermal infiltrate. Most often idiopathic, a subset of linear IgA bullous disease is induced by drugs, and intravenous vancomycin is the best-documented drug that triggers it (71). The diagnosis can be confirmed by direct immunofluorescence (72,73). In some cases the blisters resolve only after withdrawal of therapy and in others glucocorticoid therapy is required. 

The cadherin desmoglein was first identified by an unusual, but revealing, skin disease called pemphi-iU gus vulgaris, an autoimmune disease. Patients with autoimmune disorders synthesize antibodies that bind to a normal body protein. In this case, the autoantibodies disrupt adhesion between epithelial cells, causing blisters of the skin and mucous membranes. The predominant autoantibody was shown to be specific for desmoglein indeed, the addition of such antibodies to normal skin induces the formation of blisters and disruption of cell adhesion.I... 

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