BP180 and BP230 (Bullous Pemphigoid), IgG Antibodies

This test is a preferred screening test for patients suspected to have bullous pemphigoid and its variants. The antibody levels can be used to monitor the effectiveness of drug treatment.

The diverse spectrum of pemphigoid diseases is characterized by the formation of subepidermal blisters, which can develop in both the skin and mucous membranes. These conditions involve circulating autoantibodies that specifically target components found in the dermal-epidermal junction. Bullous pemphigoid (BP) presents with tense, swollen blisters that can appear on either inflamed or non-inflamed skin, with mucous membranes being rarely affected. The patient's serum contains IgG antibodies that primarily target hemidesmosomal proteins, namely BP180 and BP230. BP180 is a transmembrane glycoprotein with its primary immunogenic epitopes located in the extracellular 16th non-collagenous domain (BP180-NC16 A).

Pemphigoid gestationis is a variant of BP that occurs in pregnant women and during the postpartum period, resulting in the development of urticarial plaques and/or tense blisters. Mucous membrane pemphigoid affects one or more mucous membranes such as the oral, ocular, genital and anal regions, and can also involve the skin. Patients with this condition exhibit low-titer IgG/IgA autoantibodies directed against components of the basement membrane zone, with BP180 and laminin 332 being the two primary targets.