IGG4 FITC, IFA
Overview
USEFUL FORConfirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
METHOD NAMEIndirect Immunofluorescence
REPORTING NAMECutaneous Immfluor. Ab, S (IgG)ALIASESBasement 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
Specimen
SPECIMEN TYPESerum
SPECIMEN REQUIREDCollection Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Submission Container/Tube: Plastic vial
Specimen Volume: 2 mL
SPECIMEN MINIMUM VOLUME0.5 mL
Clinical Info
USEFUL FORConfirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
CLINICAL INFORMATIONIgG anti-basement membrane zone (BMZ) antibodies are produced by patients with pemphigoid. 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. Sensitivity of detection of anti-BMZ antibodies is increased when serum is tested using sodium chloride-split primate skin as substrate.
Circulating IgG anti-BMZ antibodies are also detected in patients with epidermolysis bullosa acquisita and bullous eruption of lupus erythematosus.
IgG anti-cell surface (CS) antibodies are produced by patients with pemphigus. The titer of anti-CS antibodies generally correlates with disease activity of pemphigus.
REFERENCE VALUESReport includes presence and titer of circulating antibodies. If serum contains basement zone (BMZ) antibodies on split-skin substrate, patterns will be reported as:
1) Epidermal pattern, consistent with pemphigoid
2) Dermal pattern, consistent with epidermolysis bullosa acquisita
Negative in normal individuals
INTERPRETATIONIndirect immunofluorescence (IF) testing may be diagnostic when histologic or direct IF studies are only suggestive, nonspecific, or negative.
Anti-cell surface (CS) antibodies correlate with a diagnosis of pemphigus.
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).
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.