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HelpTest Code RSBV Rare Subepithelial Autoimmune Blistering Disease Variants, Serum
Specimen Required
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 2 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
Useful For
Aiding in the diagnosis of rare subepithelial autoimmune blistering diseases, including anti-laminin 332 pemphigoid, anti-p200 pemphigoid, epidermolysis bullosa acquisita, and systemic bullous lupus erythematosus
Method Name
Indirect Immunofluorescence
Reporting Name
Rare Subepi Blistering Variants, SSpecimen Type
SerumSpecimen Minimum Volume
0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 14 days | |
| Frozen | 30 days | ||
| Ambient | 14 days |
Reference Values
Normal patients: Negative
Interpretation
This test panel is comprised of cell-based assays to detect antibodies directed against laminin-332, p200, or collagen VII. This panel's intended in vitro use is as an aid in the diagnosis of rare subepithelial autoimmune blistering diseases, including anti-laminin 332 pemphigoid, anti-p200 pemphigoid, epidermolysis bullosa acquisita, and systemic bullous lupus erythematosus.
A positive test result for laminin-332 antibodies may correlate with a diagnosis of laminin-332 pemphigoid in the appropriate clinical setting. Laminin-332 pemphigoid is associated with a higher rate of associated malignancy and ocular mucosal disease than conventional pemphigoid.
A positive test result for p200 antibodies may correlate with a diagnosis of p200 pemphigoid in the appropriate clinical setting. P200 pemphigoid can be associated with a more recalcitrant disease course than conventional pemphigoid and may be associated with psoriasis.
A positive test result for collagen VII antibodies may correlate with a diagnosis of epidermolysis bullosa acquisita (EBA) or bullous systemic lupus erythematosus (BSLE) in the appropriate clinical setting. EBA can be associated with inflammatory bowel disease and a more recalcitrant disease course in some patients. BSLE is usually associated with systemic lupus erythematosus.
Recommend correlation with clinical presentation, histopathologic findings from standard biopsy, direct immunofluorescence from a perilesional biopsy (CIB / Cutaneous Direct Immunofluorescence Assay, Varies), indirect immunofluorescence with IgG (CIFS / Cutaneous Immunofluorescence Antibodies, IgG, Serum), and other testing as clinically indicated.
Clinical Reference
1. Goletz S, Probst C, Komorowski L, et al. A sensitive and specific assay for the serological diagnosis of antilaminin 332 mucous membrane pemphigoid. Br J Dermatol. 2019;180(1):149-156
2. Amber KT, Bloom R, Hertl M. A systematic review with pooled analysis of clinical presentation and immunodiagnostic testing in mucous membrane pemphigoid: association of anti-laminin-332 IgG with oropharyngeal involvement and the usefulness of ELISA. J Eur Acad Dermatol Venereol. 2016;30(1):72-77. doi:10.1111/jdv.13397
3. Ahmed AR, Kalesinskas M, Kooper-Johnson S. Paraneoplastic autoimmune Laminin-332 syndrome (PALS): Anti-Laminin-332 mucous membrane pemphigoid as a prototype. Autoimmun Rev. 2023;22(10):103444. doi:10.1016/j.autrev.2023.103444
4. Seta V, Aucouturier F, Bonnefoy J, et al. Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA). J Am Acad Dermatol. 2016;74(6):1166-1172. doi:10.1016/j.jaad.2016.01.005
5. Kridin K, Kneiber D, Kowalski EH, Valdebran M, Amber KT. Epidermolysis bullosa acquisita: A comprehensive review. Autoimmun Rev. 2019 Aug;18(8):786-795. doi: 10.1016/j.autrev.2019.06.007
6. Holtsche MM Goletz S, von Georg A, et al. Serologic characterization of anti-p200 pemphigoid: Epitope spreading as a common phenomenon. J Am Acad Dermatol. 2021;84(4):1155-1157
7. Lau I, Goletz S, Holtsche MM, et al. Anti-p200 pemphigoid is the most common pemphigoid disease with serum antibodies against the dermal side by indirect immunofluorescence microscopy on human salt-split skin. J Am Acad Dermatol. 2019;81(5):1195-1197
Day(s) Performed
Varies
Report Available
1 to 7 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86255
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| RSBV | Rare Subepi Blistering Variants, S | In Process |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 621392 | Laminin 332 | In Process |
| 621393 | p200 | In Process |
| 621394 | Collagen VII | In Process |
| 621404 | Other | 77202-0 |