Bullous Pemphigoid

In the pemphigoids, IgG autoantibodies against components of the dermoepidermal basement membrane, such as bullous antigen 180 (BP180/BP antigen 2/type XVII collagen), BP antigen 230 (BP230/BP antigen 1), and laminin 5 interfere with the adhesion of basal epidermal keratinocytes to the dermoepidermal basement membrane zone (Fig. 8.1b). BP180 and BP230 are transmembranous and intracellular components, respectively, of hemidesmosomes of basal epidermal keratinocytes while laminin 5 is a ligand of BP180 located in the lamina lucida of the basement membrane zone (Fig. 8.1b). Bullous pemphigoid (BP) is the most common autoimmune blistering disease in adults. Prodromal, non-bullous periods of BP may occur in which patients demonstrate pruritic eczematiform papules or urticarial plaques. The bullous manifestations of BP present as tense, fluid-filled vesicles and bullae on normal or erythematous skin in combination with urticat-ed plaques (Fig. 8.2D-F). Involvement of the oral mucosa is rarely observed in about 10-30% of patients. In contrast to pemphigus, the use of potent topical corticosteroids such as clobetasol propionate has been shown to be effective even in the treatment of generalized BP. Nevertheless, BP patients with extensive disease are usually treated with systemic predni-sone at doses of 0.5 -1.0 mg/kg/day, which is then progressively tapered.

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