Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
Bullous pemphigoid (BP) BP180 and BP230 enzyme-linked immunosorbent assay are sensitive, objective, and specific tests that should be considered as an initial screening test in the diagnosis of pemphigoid and its variants.
To compare these results with the standard serum test of indirect immunofluorescence utilizing monkey esophagus substrate.
Enzyme-Linked Immunosorbent Assay (ELISA)
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
BP 180 and 230, S
Specimen Type Describes the specimen type needed for testing
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
Container/Tube: Red top
Specimen Volume: 1 mL
Forms: If not ordering electronically, submit a Dermatopathology/Immunodermatology Request Form (Supply T060) with the specimen.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Mild OK; Gross reject
Mild reject; Gross reject
Mild OK; Gross reject
Serum gel tube
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
|Serum Red||Refrigerated (preferred)||7 days|
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Bullous pemphigoid (BP) is chronic pruritic blistering disorder found mainly in aged persons, characterized by the development of tense blisters over an erythematous or urticarial base. IgG antibasement membrane zone antibodies are found in the serum of patients, and linear IgG and C3 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 kD and 180 kD, 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.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
<9.0 U (negative)
> or =9.0 U (positive)
<9.0 U (negative)
> or =9.0 U (positive)
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’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 biopsy, and in whom the BP180/BP230 assay is negative, follow-up testing by CIFS/8052 Cutaneous Immunofluorescence Antibodies (IgG), Serum is recommended.
Antibody titer correlates with disease activity in many patients. Patients with severe disease can usually be expected to have high titers of antibodies to BP. Titers are expected to decrease with clinical improvement.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
As with other diagnostic test procedures, the results obtained with bullous pemphigoid (BP) BP180 and BP230 enzyme-linked immunosorbent assay (ELISA) kit serve only as an aid to diagnosis and should not be interpreted as diagnostic in themselves.
Thirty-two classic bullous pemphigoid (BP), 15 mucous membrane pemphigoid, and 7 other pemphigoid variants, diagnosed by direct immunofluorescence, routine histology, and clinical presentation were tested. Controls included 47 patients with other autoimmune blistering disorders and 42 age-matched controls without skin disease. Forty of 54 (74%) patients with BP and variants tested positive for BP180 and/or BP230 autoantibodies. Of these patients, 28 of 32 (88%) with classical BP, 8 of 15 (53%) with mucous membrane predominant (MMP), and 4 of 7 (57%) of other pemphigoid variants, tested positive.
The calculated sensitivities in classical BP were 54% for BP180 alone and 56% for BP230 alone. The sensitivity increased to 88% with both tests combined, which is comparable to that of indirect immunofluorescence (IIF) (88%). In MMP the calculated sensitivities were 47% for BP180 alone, 13% for BP230 alone, and 53% for both combined. This was slightly less than the sensitivity of IIF (67%). Only 5 of 47 (11%) and 2 of 47 (4%) control patients with other autoimmune blistering disorders were positive for BP180 and BP230 autoantibodies respectively. Interestingly, the 2 patient’s positive for BP230 autoantibody had paraneoplastic pemphigus. One of 42 (2%) and 0 normal controls tested positive for BP180 and BP230 respectively.
The calculated specificities for BP180, BP230, and IIF were 93%, 98%, and 92% respectively.
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
1. Liu Z, Diaz LA, Troy JL: A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest 1993;92:2480-2488
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 Dematol Res 1996;288:507-509
3. Stanley JR, Hawley-Nelson P, Yuspa SH, et al: Characterization of bullous pemphigoid antigen: a unique basement membrane protein of stratified aqueous epithelia. Cell 1981;24:897-903
4. Hamada T, Nagata Y, Tmita M, et al: 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;10:256-263
Method Description Describes how the test is performed and provides a method-specific reference
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.(Unpublished Mayo method)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday through Friday; 7 a.m.-5 p.m.
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
Same day/1 day (Samples are tested 1 day/week)
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
Test Classification Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
This test has been cleared or approved by the U.S. Food and Drug Administration and is used per manufacturer's instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements.
CPT Code Information Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
83516 x 2
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
|Result ID||Reporting Name||LOINC Code|
|88874||BP 180, S||53842-1|
|28259||BP 230, S||53843-9|