Test ID: DSG13
Desmoglein 1 (DSG1) and Desmoglein 3 (DSG3), Serum
Secondary ID 
A test code used for billing and in test definitions created prior to November 2011
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
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)
As an aid in the diagnosis of pemphigus
Monitoring treatment response in patients with a confirmed diagnosis of pemphigus
Method Name A short description of the method used to perform the test
Enzyme-Linked Immunosorbent Assay (ELISA)
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Specimen Type Describes the specimen type needed for testing
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Specimen Volume: 1 mL
Forms: If not ordering electronically, submit a Dermatopathology/Immunodermatology Request Form (Supply T060) with the specimen.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Reject Due To Identifies specimen types and conditions that may cause the specimen to be rejected
| Hemolysis | Mild OK; Gross reject |
| Lipemia | Mild reject; Gross reject |
| Icterus | Mild OK; Gross reject |
| Other | NA |
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
| Specimen Type | Temperature | Time |
|---|---|---|
| Serum | Refrigerated (preferred) | 7 days |
| Frozen | 14 days | |
| Ambient | 36 hours |
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 monkey 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 (ELISA) 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
<14.0 U (negative)
14.0-20.0 U (indeterminate)
>20.0 U (positive)
DESMOGLEIN 3
<9.0 U (negative)
9.0-20.0 U (indeterminate)
>20.0 U (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, the IIF test (CIFS/8052 Cutaneous Immunofluorescence Antibodies [IgG], Serum) is recommended.
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 drawn 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 Provides recommendations for further in-depth reading of a clinical nature
1. Amagai M, Tsunoda K, Zillikens D, et al: The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am 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, Gratin MJ, Bhogal SJ, et al: The clinical significance of autoantibodies to desmoglein 1 in 78 cases of pemphigus vulgaris. J Invest Derm 1999;115:568
Method Description Describes how the test is performed and provides a method-specific reference
This ELISA method detects and measures serum levels of antibodies of certain pemphigus diseases. Calibrators and patient sera are added to microwells coated with DSG1 and 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. (Ishii K, Amagai M, Hall RP, et al: Characterization of autoantibodies in pemphigus using antigen specific ELISA’s with baculovirus expresses recombinant desmogleins. J Immunol 1997 Aug 15;159[4]:2010-2017)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday; 8 a.m.
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
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's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
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 Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
83516 x 2
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
| Result ID | Reporting Name | LOINC Code |
|---|---|---|
| 83680 | DSG 1 | 43311-0 |
| 24027 | DSG 3 | 43312-8 |
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