Bullous pemphigoid with the deposition of IgG2 but not IgG1, IgG3 nor IgG4 autoantibodies at the basement membrane zone

Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease, characterized by tense bullae and urticarial erythema with a generalized distribution. BP is induced by autoantibodies to structural proteins of the basement membrane zone (BMZ) such as BP180 and BP230¹. Because the majority of BP skin shows complement deposition², complement activation is considered to be important for blister formation. Among IgG subclasses, IgG1 have a high ability to activate complements, whereas IgG2 and IgG4 have a low and no ability, respectively.

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