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HelpTest Code CIFS Cutaneous Immunofluorescence Antibodies, IgG and IgG4, Serum
Reporting Name
Cutaneous Immfluor. Ab, IgG/IgG4, SUseful For
Confirming the presence of IgG and/or IgG4 antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Specimen Type
SerumSpecimen Required
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.
Specimen Minimum Volume
0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time |
|---|---|---|
| Serum | Refrigerated (preferred) | 14 days |
| Frozen | 30 days | |
| Ambient | 14 days |
Reference Values
Negative in normal individuals
Day(s) Performed
Monday through Friday
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
88346
88350
88350 (if appropriate)
LOINC Code Information
| 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 |
|---|---|---|
| 21539 | Cell Surface Ab IgG | 93233-5 |
| 21540 | Basement Membrane IgG | 29994-1 |
| 21542 | Primate Split Skin IgG | 104832-1 |
| 622319 | Cell Surface Ab IgG4 | In Process |
| 622320 | Basement Membrane IgG4 | In Process |
| 622322 | Primate Split Skin IgG4 | In Process |
| 21638 | Other | 48767-8 |
Clinical Information
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.
Interpretation
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
Method Description
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)
Reject Due To
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
Method Name
Indirect Immunofluorescence Assay (IFA)
Secondary ID
8052Reflex Tests
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| CIFST | CIFS Titer, IgG, S | No | No |
Testing Algorithm
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.