Confirming the presence of IgA antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
Indirect Immunofluorescence Assay (IFA)
Basement Membrane Antibodies
Bullous Lupus Erythematosus
Bullous Pemphigoid
Cicatricial Pemphigoid
CII, Serum
Circulating IgA 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
Lupus Erythematosus (LE)
Pemphigoid
Pemphigus
Skin Basement Membrane Antibodies
Split-Skin Assay
Serum
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.
0.5 mL
Gross hemolysis | OK |
Gross lipemia | Reject |
Gross icterus | OK |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 14 days | |
Frozen | 30 days | ||
Ambient | 14 days |
Confirming the presence of IgA antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
Immunoglobulin A anti-basement membrane zone (BMZ) antibodies are produced by patients with pemphigoid. In most patients with bullous pemphigoid, serum contains IgA anti-BMZ antibodies, while in cicatricial pemphigoid circulating IgA 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 IgA anti-BMZ antibodies are also detected in patients with epidermolysis bullosa acquisita and bullous eruption of lupus erythematosus.
IgA anti-cell surface (CS) antibodies are produced by patients with pemphigus. The titer of anti-CS antibodies generally correlates with disease activity of pemphigus.
Report includes presence and titer of circulating antibodies. If serum contains basement membrane zone 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
Indirect immunofluorescence (IF) testing may be diagnostic when histologic or direct IF studies are only suggestive, nonspecific, or negative.
Anti-cell surface 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.
Results should be interpreted in conjunction with clinical information, histologic pattern, and results of direct immunofluorescence (IF) study. In particular, the finding of low titer (< or =1:80) anti-CS antibodies should not be used alone (ie, without histologic or direct IF support) to confirm a diagnosis of pemphigus.
1. Caux F, Kirtschig G, Lemarchand-Venencie F, et al. IgA-epidermolysis bullosa acquisita in a child resulting in blindness. Br J Dermatol. 1997;137(2):270-275
2. Chorzelski TP, Jablonska S. IgA linear dermatosis of childhood (chronic bullous disease of childhood). Br J Dermatol. 1979;101(5):535-542
3. Guide SV, Marinkovich MP. Linear IgA bullous dermatosis. Clin Dermatol. 2001;19(6):719-727
4. Hashimoto T, Ebihara T, Nishikawa T. Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. J Dermatol Sci. 1996;12(1):10-17
5. Lally A, Chamberlain A, Allen J, Dean D, Wojnarowska F. Dermal-binding linear IgA disease: an uncommon subset of a rare immunobullous disease. Clin Exp Dermatol. 2007;32(5):493-498
6. Tsuruta D, Ishii N, Hamada T, et al. IgA pemphigus. Clin Dermatol. 2011;29(4):437-442
7. Vodegel RM, de Jong MCJM, Pas HH, Jonkman MF. IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature. J Am Acad Dermatol. 2002;47(6):919-925
8. Willsteed E, Bhogal BS, Black MM, McKee P, Wojnarowska F. Use of 1M NaCl split skin in the indirect immunofluorescence of the linear IgA bullous dermatoses. J Cutan Pathol. 1990;17(3):144-148
9. Wilson BD, Beutner EH, Kumar V, Chorzelski TP, Jablonska S. Linear IgA bullous dermatosis. An immunologically defined disease. Int J Dermatol. 1985;24(9):569-574
10. Wojnarowska F, Collier PM, Allen J, Millard PR. The localization of the target antigens and antibodies in linear IgA disease is heterogeneous, and dependent on the methods used. Br J Dermatol. 1995;132(5):750-757
11. Tirumalae R, Kalegowda IY. Role of BIOCHIP indirect immunofluorescence test in cutaneous vesiculobullous diseases. Am J Dermatopathol. 2020;42(5):322-328
Frozen sections of primate esophagus and sodium chloride-split primate skin are overlaid with dilutions of patient's serum, incubated, covered with fluorescein-conjugated IgA antiserum, and interpreted with 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
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
CIFA | Cutaneous Immfluor. Ab (IgA), S | 104828-9 |
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.
|
---|---|---|
610628 | Cell Surface Ab IgA | 104829-7 |
610629 | Basement Membrane IgA | 104830-5 |
610630 | Primate Esophagus IgA | 104833-9 |
610631 | Primate Split Skin IgA | 104834-7 |
610632 | Other | 48767-8 |