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Test Code BPAB Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum


Specimen 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.


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)

Method Name

Enzyme-Linked Immunosorbent Assay (ELISA)

Reporting Name

BP 180 and 230, Serum

Specimen Type

Serum Red

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

Reference Values

BULLOUS PEMPHIGOID 180:

<20 RU/mL (negative)

≥20 RU/mL (positive)

 

BULLOUS PEMPHIGOID 230:

<20 RU/mL (negative)

≥20 RU/mL (positive)

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.

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

Day(s) Performed

Varies

Report Available

1 to 5 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test 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.

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