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Test Code CIFA Cutaneous Immunofluorescence Antibodies, IgA, Serum


Specimen Required


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.


Secondary ID

610627

Useful For

Confirming the presence of IgA antibodies to diagnose pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus

Method Name

Indirect Immunofluorescence Assay (IFA)

Reporting Name

Cutaneous Immfluor. Ab (IgA), S

Specimen Type

Serum

Specimen 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

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Clinical Information

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.

Reference Values

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

Interpretation

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.

Cautions

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 (≤1:80) anti-CS antibodies should not be used alone (ie, without histologic or direct IF support) to confirm a diagnosis of pemphigus.

Clinical Reference

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

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 IgA antiserum, and interpreted with a fluorescence microscope.(Unpublished Mayo method)

Day(s) Performed

Monday through Friday

Report Available

2 to 7 days

Specimen Retention Time

30 days

Performing 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

88346

88350

LOINC Code Information

Test ID Test Order Name Order LOINC Value
CIFA Cutaneous Immfluor. Ab (IgA), S 104828-9

 

Result ID Test Result Name Result LOINC Value
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