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HelpEpic Code LAB1230576 Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum
Additional Codes
Mayo code: BPAB
Interface code: 1230576
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Useful For
Initial screening test in the diagnosis of bullous pemphigoid and its variants
Complementing the standard serum test of indirect immunofluorescence utilizing primate esophagus substrate and primate salt-split skin substrate (CIFS / Cutaneous Immunofluorescence Antibodies [IgG], Serum)
Specimen Type
Serum RedSpecimen Required
Collection Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Submission container/tube: Plastic vial
Specimen Volume: 1 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
COLLECTION NOTE: Volumes listed are in serum or plasma, draw approximately 2 1/2 times the requested volume in whole blood.
Specimen Minimum Volume
0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum Red | Refrigerated (preferred) | 14 days | |
| Frozen | 30 days | ||
| Ambient | 14 days |
Reject Due To
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
Day(s) Performed
Varies
Reference Values
BULLOUS PEMPHIGOID 180:
<20 RU/mL (negative)
≥20 RU/mL (positive)
BULLOUS PEMPHIGOID 230:
<20 RU/mL (negative)
≥20 RU/mL (positive)
Clinical Information
Bullous pemphigoid (BP) is a chronic pruritic blistering disorder found mainly in aged persons, characterized by the development of tense blisters over an erythematous or urticarial base. IgG anti-basement membrane zone antibodies are found in the serum of patients, and linear IgG and complement component 3 sediment is found on the basement membrane zone of the lesion. Several well characterized variants exist including localized, mucous membrane predominant and pemphigoid gestationis, also referred to as herpes gestationis.
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 kDa and 180 kDa, respectively. BP180 is thought to be the direct target of the autoantibody because of its location along the basement membranes, and the autoantibody against BP230 is thought to be secondarily produced.
Cautions
As with other diagnostic test procedures, the results obtained with bullous pemphigoid (BP) BP180 and BP230 enzyme-linked immunosorbent assay kit serve only as an aid to diagnosis and should not be interpreted as diagnostic in themselves.
Performance of the assay in pediatric patients has not been established.
Performance of the assay on other matrices besides serum has not been established.
Interpretation
Antibodies to bullous pemphigoid (BP) BP180 and BP230 have been shown to be present in most patients with pemphigoid. Adequate sensitivities and specificity for disease are documented and Mayo Clinic's experience demonstrates a very good correlation between BP180 and BP230 results and the presence of pemphigoid (see Supportive Data). However, in those patients strongly suspected to have pemphigoid, either by clinical findings or by routine biopsyor direct immunofluorescence, and in whom the BP180/BP230 assay is negative, follow-up testing by CIFS / Cutaneous Immunofluorescence Antibodies (IgG), Serum is recommended.
Antibody titer may correlate with disease activity in some patients. Patients with severe disease may be expected to have high titers of antibodies to BP. Titers may decrease with clinical improvement.
Reporting Name
BP 180 and 230, SerumMethod Name
Enzyme-Linked Immunosorbent Assay (ELISA)
Method Description
This enzyme-linked immunosorbent assay (ELISA) method detects and measures serum levels of antibodies of certain pemphigoid diseases. Calibrators and patient sera are added to microwells coated with bullous pemphigoid (BP) BP180 and BP230 antigens, allowing antibodies to react with the immobilized antigens. After washing to remove any unbound serum proteins, horseradish peroxidase-conjugated IgG is added and incubated. Following another wash step, the peroxidase substrate is added and allowed to incubate for an additional period. Stop solution is then added to each well to cancel the enzyme reaction and to stabilize the color development. The assay can be quantified by measuring the reaction photometrically and plotting the results. The amount of antigen specific bound antibody is proportional to the color intensity.(Package inserts: Anti-BP180-NC16A-4X ELISA [IgG], Form EA_1502-2G_A_US_D04. EuroImmun; 07/08/2020; Anti-BP230-CF ELISA [IgG], Form EA_1502-1G_A_UK_C03.doc. EuroImmun; 10/23/2020)
CPT Code Information
83516 x 2
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| BPAB | BP 180 and 230, Serum | 92671-7 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 606816 | BP 180, S | 53842-1 |
| 606817 | BP 230, S | 53843-9 |
Report Available
1 to 5 daysTest Classification
This test has been modified from the manufacturer's instructions. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.Clinical Reference
1. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest. 1993;92(5):2480-2488
2. Matsumura K, Amagai M, Nishikawa T, Hashimoto T. The majority of bullous pemphigoid and herpes gestationes serum samples react with the NC16a domain of the e180-kD bullous pemphigoid antigen. Arch Dermatol Res. 1996;288(9):507-509
3. Stanley JR, Hawley-Nelson P, Yuspa SH, Shevach EM, Katz SI. Characterization of bullous pemphigoid antigen: a unique basement membrane protein of stratified aqueous epithelia. Cell. 1981;24(3):897-903
4. Hamada T, Nagata Y, Tomita M, Salmhofer W, Hashimoto T. Bullous pemphigoid sera react specially with various domains of BP230, most frequently with C-terminal domain, by immunblot analyses using bacterial recombinant proteins covering the entire molecule. Exp Dermatol. 2001;10(4):256-263
5. Rico MJ, Korman NJ, Stanley JR, Tanaka T, Hall RP. IgG antibodies from patients with bullous pemphigoid bind to localized epitopes on synthetic peptides encoded by bullous pemphigoid antigen cDNA. J Immunol. 1990;145(11):3728-3733
6. Wieland CN, Comfere NI, Gibson LE, Weaver AL, Krause PK, Murray JA. Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaffected subjects. Arch Dermatol. 2010;146(1):21-25
7. Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS. Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol. 2021;85(1):1-14
8. Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN. Subepithelial autoimmune bullous dermatoses: disease activity assessment and therapy. J Am Acad Dermatol. 2021;85(1):18-27