Test Catalog

Test Id : DSGAB

Desmoglein 1 (DSG1) and Desmoglein 3 (DSG3), IgG Antibodies, Serum

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

Preferred screening test for patients suspected to have an autoimmune blistering disorder of the skin or mucous membranes (pemphigus)

 

Aiding in the diagnosis of pemphigus

Method Name
A short description of the method used to perform the test

Enzyme-Linked Immunosorbent Assay (ELISA)

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

Desmoglein 1 and 3, Serum

Aliases
Lists additional common names for a test, as an aid in searching

DSG1

DSG3

Bullous Pemphigus

Specimen Type
Describes the specimen type validated for testing

Serum

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

0.5 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 14 days
Frozen 30 days
Ambient 14 days

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

Preferred screening test for patients suspected to have an autoimmune blistering disorder of the skin or mucous membranes (pemphigus)

 

Aiding in the diagnosis of pemphigus

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

Pemphigus includes a group of often fatal autoimmune blistering diseases characterized by intraepithelial lesions. Pemphigus vulgaris and its variants may present with oral or mucosal lesions alone or with mucosal plus skin lesions. Pemphigus foliaceous and variants present with skin lesions alone.

 

Indirect immunofluorescence (IIF) studies reveal that both forms of pemphigus are caused by autoantibodies to cell surface antigens of stratified epithelia or mucous membranes and skin. These antibodies bind to calcium-dependent adhesion molecules in cell surface desmosomes, notably desmoglein 1 (DSG1) in pemphigus foliaceus and desmoglein 3 (DSG3) and/or DSG1 in pemphigus vulgaris. Desmogleins are protein substances located in and on the surface of keratinocytes. These proteins have been shown to be a critical factor in cell-to-cell adhesion. Antibodies to desmogleins can result in loss of cell adhesion, the primary cause of blister formation in pemphigus.

 

The diagnosis of pemphigus depends on biopsy and serum studies that characterize lesions and detect the autoantibodies that cause them. Originally, the serum studies were performed by IIF using primate esophagus and other tissue substrates. The identification of the reactive antigens as DSG1 and DSG3 has made it possible to develop highly specific and sensitive enzyme-linked immunosorbent assay methods.

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.

DESMOGLEIN 1:

<20 RU/mL (negative)

> or =20 RU/mL (positive)

 

DESMOGLEIN 3:

<20 RU/mL (negative)

> or =20 RU/mL (positive)

Interpretation
Provides information to assist in interpretation of the test results

Antibodies to desmoglein 1 (DSG1) and desmoglein 3 (DSG3) have been shown to be present in patients with pemphigus. Many patients with pemphigus foliaceus, a superficial form of pemphigus have antibodies to DSG1. Patients with pemphigus vulgaris, a deeper form of pemphigus, have antibodies to DSG3 and sometimes DSG1 as well.

 

Antibody titer correlates in a semiquantitative manner with disease activity in many patients. Patients with severe disease can usually be expected to have high titers of antibodies to DSG. Titers are expected to decrease with clinical improvement.

 

Our experience demonstrates a very good correlation between DSG1 and DSG3 results and the presence of pemphigus. Adequate sensitivities and specificity for disease are documented. However, in those patients strongly suspected to have pemphigus either by clinical findings or by routine biopsy, and in whom the DSG assay is negative, indirect immunofluorescence testing is recommended. For more information see CIFS / Cutaneous Immunofluorescence Antibodies [IgG], Serum.

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

Recommend repeat testing of indeterminate specimens, either with a fresh specimen collected at a later time or the original specimen tested by another method.

 

The desmoglein 1 (DSG1) and desmoglein 3 (DSG3) results serve only as an aid to diagnosis and should not be interpreted as diagnostic by themselves. The results should be interpreted in conjunction with clinical evaluation of the patient along with other diagnostic procedures.

 

Performance of these assays in the pediatric population has not been established.

 

The assay performance characteristics have not been established for matrices other than serum.

 

A positive result indicates the presence of antibodies to recombinant DSG1 and DSG3 and does not specifically identify a certain type of pemphigus.

 

A negative result does not rule out the presence of pemphigus.

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

1. Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T: The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999 Feb;40(2 Pt 1):167-170

2. Amagai M, Komai A, Hashimoto T, et al: Usefulness of enzyme-linked immunoabsorbent assay using recombinant desmogleins 1 and 3 for sero-diagnosis of pemphigus. Brit J Dermatol. 1999 Feb;140(2):351-357

3. Harman KE, Gratian MJ, Bhogal BS, Challacombe SJ, Black M: The clinical significance of autoantibodies to desmoglein 1 in 78 cases of pemphigus vulgaris. J Invest Derm. 1999;112(4):568. Abstract 273

4. Harman KE, Gratian MJ, Seed PT, Bhogal BS, Challacombe SJ, Black MM: Diagnosis of pemphigus by ELISA: a critical evaluation of two ELISAs for the detection of antibodies to the major pemphigus antigens, desmoglein 1 and 3. Clin Exp Dermatol. 2000 May;25(3):236-240

5. Prussmann W, Prussmann J, Koga H, et al: Prevalence of pemphigus and pemphigoid autoantibodies in the general population. Orphanet J Rare Dis. 2015 May 15;10:63

6. Toosi S, Collins JW, Lohse CM, et al: Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus. Int J Dermatol. 2016 Apr;55(4):e184-e190

7. Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS: Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol. 2021 Jun;84(6):1507-1519

Method Description
Describes how the test is performed and provides a method-specific reference

This enzyme-linked immunosorbent assay (ELISA) method detects and measures serum levels of antibodies of certain pemphigus diseases. Calibrators and patient sera are added to microwells coated with desmoglein 1 (DSG1) and desmoglein 3 (DSG3) antigens, allowing antibodies to react with the immobilized antigens. After washing to remove any unbound serum proteins, horseradish peroxidase-conjugated IgG is added and incubated. Following another wash step, the peroxidase substrate is added and allowed to incubate for an additional period. Stop solution is then added to each well to cancel the enzyme reaction and to stabilize the color development. The assay can be quantified by measuring the reaction photometrically and plotting the results. The amount of antigen specific bound antibody is proportional to the color intensity.(Package inserts: Anti-Desmoglein 1 ELISA [IgG], Form EA_1495G_A_US_D03.doc. EuroImmun; Version: 7/7/11; Anti-Desmoglein 3 ELISA [IgG], Form EA_1496G_A_US_D03.doc. EuroImmun; Version: 7/7/11)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Varies

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

1 to 10 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

30 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

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  • Prospective clients should contact their Regional Manager. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

This test has been modified from the manufacturer's instructions. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

83516 x 2

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
DSGAB Desmoglein 1 and 3, Serum 94335-7
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.
606818 DSG 1 94336-5
606819 DSG 3 94337-3

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports