Anti–BP180-NC16A-4X Antibodies

Autoimmune blistering diseases are organ-specific autoimmune conditions characterised by autoantibodies against structural skin proteins. They are divided into four main groups depending on target antigens and clinical presentation: pemphigoid diseases, pemphigus diseases—including epidermolysis bullosa acquisita (EBA)—paraneoplastic pemphigus, and dermatitis herpetiformis (DH).

Diagnosis and differentiation of autoimmune blistering diseases rely on clinical assessment combined with the detection of autoantibodies against specific target antigens.

Bullous pemphigoid (BP) is the most common autoimmune blistering dermatosis, mainly affecting older adults  and manifests with tense blisters on the skin.

Autoantibodies in BP are directed against two hemidesmosomal proteins: BP180 (type XVII collagen) and BP230.

BP180 is a transmembrane glycoprotein with an intracellular C-terminal and an extracellular N-terminal region. Its ectodomain consists of 15 collagenous and 16 non-collagenous domains. The 16^th non-collagenous domain (NC16A), adjacent to the keratinocyte membrane, represents the immunogenic epitope. The majority of BP patients present antibodies against BP180 NC16A. The BP180-NC16A region is also immunodominant in pemphigoid gestationis and lichen planus pemphigoides, and is present in about half of mucous membrane pemphigoid cases.

Serum levels of anti-BP180 autoantibodies correlate with BP disease activity, whereas anti-BP230 levels correlate with disease duration. Consequently, their measurement enables an appropriate and reliable serological identification of BP and helps assess disease activity before and during therapy.