Confirming the presence of IgG and/or IgG4 antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
Test Id | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
CIFST | CIFS Titer, IgG, S | No | No |
If IgG anti-cell surface or anti-basement membrane zone antibodies are present, then the IgG antibody titer will be reflexed and performed at an additional charge.
Indirect Immunofluorescence Assay (IFA)
Basement Membrane Antibodies
Bullous Pemphigoid
Cicatricial Pemphigoid
CII, Serum
Circulating IgG Basement Membrane Zone Antibody
Circulating Intercellular Ab Study
Epidermal Cell Surface Antibody
Epidermal Fluorescence Antibody
Epidermolysis Bullosa Acquisita
Epithelial Antibody
Herpes gestationis
IMF - Epidermal Fluorescent Ab
Indirect Immunofluorescence
Intercellular Substance Antibody
Pemphigoid
Pemphigus
Skin Basement Membrane Antibodies
Split-Skin Assay
Lupus Erythematosus (LE)
Bullous Lupus Erythematosus
If IgG anti-cell surface or anti-basement membrane zone antibodies are present, then the IgG antibody titer will be reflexed and performed at an additional charge.
Serum
Collection Container/Tube:
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
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.
0.5 mL
Gross hemolysis | OK |
Gross lipemia | OK |
Gross icterus | OK |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Ambient | 14 days | |
Refrigerated (preferred) | 14 days | ||
Frozen | 30 days |
Confirming the presence of IgG and/or IgG4 antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
If IgG anti-cell surface or anti-basement membrane zone antibodies are present, then the IgG antibody titer will be reflexed and performed at an additional charge.
Immunoglobulin G (IgG) and/or IgG4 anti-basement membrane zone (BMZ) antibodies are produced by patients with pemphigoid, pemphigus, and other rare autoimmune blistering disorders such as epidermolysis bullosa acquisita and bullous lupus erythematosus. In most patients with bullous pemphigoid, serum contains IgG anti-BMZ antibodies, while in cicatricial pemphigoid circulating IgG anti-BMZ antibodies are found in a minority of cases.
Circulating IgG4 is also variably present. Sensitivity of detection of anti-BMZ antibodies is increased when serum is tested using both sodium chloride-split primate skin and primate esophagus as substrates and using both IgG and IgG4 reactants.
Negative in normal individuals
Indirect immunofluorescence (IF) testing may aid in the diagnosis of these conditions when correlated with histopathologic, direct immunofluorescence, and clinical information.
Anti-basement membrane zone (BMZ) antibodies correlate with a diagnosis of bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita (EBA), or bullous eruption of lupus erythematosus (LE).
Anti-cell surface antibodies correlate with a diagnosis of pemphigus.
If serum contains anti-BMZ antibodies, the pattern of fluorescence on sodium chloride (NaCl)-split skin substrate helps distinguish pemphigoid from EBA and bullous LE. Staining of the roof (epidermal side) or both epidermal and dermal sides of NaCl-split skin correlates with the diagnosis of pemphigoid, while fluorescence localized only to the dermal side of the split-skin substrate correlates with either EBA or bullous LE.
The report includes presence and titer of circulating antibodies.
If the serum contains BMZ antibodies on the split-skin substrate, patterns will be reported as one of the following:
1. Epidermal pattern, suggestive of pemphigoid
2. Dermal pattern, suggestive of epidermolysis bullosa acquisita, bullous lupus erythematosus, or rare pemphigoid variants
3. Mixed pattern, suggestive of rare pemphigoid variants
If serum contains BMZ antibodies on the primate esophagus substrate, patterns will be reported as one of the following:
-BMZ (linear) pattern, suggestive of a subepidermal autoimmune mucocutaneous blistering disorder
-Intercellular substance (cell-surface) pattern, suggestive of pemphigus
Results should be interpreted in conjunction with clinical information, histopathologic pattern, and results of direct immunofluorescence (DIF) study. In particular, the finding of low titer (< or =1:80) antibodies should not be used alone (ie, without histopathologic or DIF support) to diagnose these conditions.
1. Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. Wiley Medical Publication; 1987
2. Gammon WR, Briggaman RA, Inman AO 3rd, Queen LL, Wheeler CE. Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol. 1984;82(2):139-144
3. Tirumalae R, Kalegowda IY. Role of BIOCHIP indirect immunofluorescence test in cutaneous vesiculobullous diseases. Am J Dermatopathol. 2020;42(5):322-328
4. Lehman JS, Aghazadeh Mohandesi N, Agrawal S, et al. Indirect immunofluorescence testing for immunoglobulin G4 and IgG increases test sensitivity over that of IIF testing for IgG alone for pemphigoid and pemphigus diseases: A retrospective study of 278 cases. J Am Acad Dermatol. 2024:S0190-9622(24)03372-3
Frozen sections of primate esophagus and sodium chloride-split primate skin are overlaid with dilutions of patient's serum, incubated, covered with fluorescein-conjugated IgG and IgG4 anti-serum, and interpreted using a fluorescence microscope.(Unpublished Mayo method)
Monday through Friday
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.
88346
88350
88350 (if appropriate)
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
CIFS | Cutaneous Immfluor. Ab, IgG/IgG4, S | In Process |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
21539 | Cell Surface Ab IgG | 93233-5 |
21540 | Basement Membrane IgG | 29994-1 |
21542 | Primate Split Skin IgG | 104832-1 |
21638 | Other | 48767-8 |
622319 | Cell Surface Ab IgG4 | In Process |
622320 | Basement Membrane IgG4 | In Process |
622322 | Primate Split Skin IgG4 | In Process |
Change Type | Effective Date |
---|---|
File Definition - Algorithm | 2025-06-10 |