IgA bullous pemphigoid: a distinct blistering disorder.

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Abstract
A patient is reported with an eccrine carcinoma who developed localized blistering, which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear Ig[immunoglobin]A deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. In adults linear IgA deposition may occur in 3 separate situations: dermatitis herpetiformis, bullous pemphigoid and a 3rd condition (this case is an example) which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma, but the association lends support to the concept that this eruption represents a variant of pemphigoid.