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


Specimen Required


Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Specimen Volume: 1 mL


Secondary ID

606818

Useful For

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

Enzyme-Linked Immunosorbent Assay (ELISA)

Reporting Name

Desmoglein 1 and 3, Serum

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 OK
Gross icterus OK

Clinical Information

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 methods.

Reference Values

DESMOGLEIN 1

<20 RU/mL (negative)

≥20 RU/mL (positive)

 

DESMOGLEIN 3

<20 RU/mL (negative)

≥20 RU/mL (positive)

Interpretation

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 indirect immunofluorescence (CIFS / Cutaneous Immunofluorescence Antibodies [IgG], Serum) is recommended.

Clinical Reference

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

4. Harman KE, Gratian MJ, Seed PT, et al: 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;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;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;55:e184-190

Day(s) and Time(s) Performed

Once or twice weekly, Monday through Friday; 7:30 a.m.- 5 p.m., days of testing to be determined by the laboratory.

Analytic Time

1 day

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

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 U.S. Food and Drug Administration.

CPT Code Information

83516 x 2

LOINC Code Information

Test ID Test Order Name Order LOINC Value
DSGAB Desmoglein 1 and 3, Serum In Process

 

Result ID Test Result Name Result LOINC Value
606818 DSG 1 43311-0
606819 DSG 3 43312-8