016
Test #
Bullous Pemphigoid (BPAG2) 180 & BP (BPAG1) 230 Antibody
CPT Code(s) #
83520 x2
If Profile, Includes Tests:
N/A
Disease Name:
Bullous Pemphigoid, Mucous Membrane Pemphigoid (MMP), Pemphigoid Gestationis
Type of Study:
Serum Studies
Methodology:
ELISA
Substrate:
N/A
Reference Range:
Ratio <1.0: Negative
Ratio ≥1.0: Positive
Units:
Ratio
Schedule:
Assay performed once every week.
Specimen Requirements:
Collect 2-5 ml of blood in a red top Vacutainer or serum separator (SST) tube, allow blood to clot, and centrifuge. Label tube appropriately.
Sample Stability:
Room temperature = stable during shipment
2ºC to 8ºC = 5 days
-25ºC to -15ºC = 1 year
Clinical Relevance:
Bullous pemphigoid (BP) is an autoimmune mediated immunobullous skin disorder found mainly in the elderly population and is characterized by frequent occurring of tense blisters and erythema. Antibodies are directed to the basement membrane zone and are frequently found in the serum of patients. Target antigens of the autoantibodies in BP patient serum are BP230 and BP180, also called BPAG1 and BPAG2. Molecular weight of these antigens is 230 kD and 180 kD respectively. BP180 is thought to be the direct target of the autoantibody because of its location, and the autoantibodies against BP230 are thought to be secondarily produced. The antibodies against BP180 are thought to be pathogenic, because the rabbit antibody against mouse in the NC16a region of BP180 forms blisters similar to BP when injected into neonatal mice. The main epitope of BP180 is located in the region close to cell membrane called NC16a and most patient serum reacts with the recombinant NC16a protein. Serum levels of BP180 co-relate with the disease activity.
