Test Code #

016

CPT Code(s) #

83516 (x2)

Test Name

Bullous Pemphigoid (BPAG2) 180 & BP (BPAG1) 230 Abs

If Profile, Includes Tests:

N/A

Disease Name:

Bullous Pemphigoid, Mucous Membrane Pemphigoid (MMP), Pemphigoid Gestationis

Type of Study:

Serum Studies

Methodology:

ELISA

Substrate:

Reference Range:

Negative: < 9 U/ml
Positive: � 9 U/ml

Units:

U/ml

Schedule:

Assay performed once every week. Report availability is within one week from the time of specimen receipt

Specimen Requirements:

Collect 5-10 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into red capped provided with Beutner Laboratories collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.

Sample Stability:

Stable at ambient temperature during shipment. If sample is stored prior to shipment, it is stable refrigerated (2-8ºC) up to five days and frozen (-20ºC or lower) up to one 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.

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