TEST CATALOG ORDERING & RESULTS SPECIMEN HANDLING CUSTOMER SERVICE EDUCATION & INSIGHTS
Test Catalog

Test ID: CIFA    
Cutaneous Immunofluorescence Antibodies, IgA, Serum

Useful For Suggests clinical disorders or settings where the test may be helpful

Confirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Immunoglobulin A anti-basement 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 (NaCl)-split human skin as substrate.

 

Circulating IgA anti-BMZ antibodies are also detected in patients with epidermolysis bullosa acquisita (EBA) 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 Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

Report 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 or 2) dermal pattern, consistent with epidermolysis bullosa acquisita.

 

Negative in normal individuals

Interpretation Provides information to assist in interpretation of the test results

Indirect 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 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 Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

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.

Clinical Reference Recommendations for in-depth reading of a clinical nature

1. Caux F, Kirtschig G, Lemarchand-Venencie F, et al: IgA-epidermolysis bullosa acquisita in a child resulting in blindness. Br J Dermatol. 1997 Aug;137(2):270-275

2. Chorzelski TP, Jablonska S: IgA linear dermatosis of childhood (chronic Bullous disease of childhood). Br J Dermatol. 1979 Nov;101(5):535-542

3. Guide SV, Marinkovich MP: Linear IgA bullous dermatosis. Clin Dermatol. Nov-Dec 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 Apr;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 Sep;32(5):493-498

6. Tsuruta D, Ishii N, Hamada T, et al: IgA pemphigus. Clin Dermatol. 2011 Jul-Aug;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 Dec;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 Jun;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 Nov;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 May;132(5):750-757