Suspected autoimmune skin disease such as pemphigoid and differential diagnosis of pemphigus and EBA.
Serum samples are analyzed by ELISA technique with recombinant BP180 and BP230.
BP180 has a molecular weight of 180kD and is also called BPAG2. BP230 has a molecular weight of 230kD and is also called BPAG1.
The result is given as positive or negative.
< 1.00 negative and ≥ 1.00 positive
Antibodies towards BP180 and BP230 occurs in patients with bladder disease Bullous pemphigoid (BP). The antibodies are directed against the two components of the basement membrane zone of the epithelium BP180 (type XVII collagen) and BP230. Anti-BP230 are found in approximately 65% of patients with BP, while the anti-BP180 are found in almost all patients.
The serum level of anti-BP180 correlates with disease activity and BP230 correlates with disease duration. However, it seems that anti-BP230 does not cause blisters but is rather a consequence of damage to the keratinocytes. The antibodies may also be associated with paraneoplastic pemphigoid and other variations of BP. The different variants of BP include Pemphigoid gestationis (PG) that can develop during pregnancy and Cicatricial pemphigoid (CP) which is a sub epidermal autoimmune bladder disease.