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Desmoglein 1 and 3 Antibodies

Item Value
Available Stat? No
Test code DSG
Performed by Mayo
Sendout? Yes
Method ELISA
Container type Red top or Gold top
Amount to Collect 2 mL blood
Sample type Serum
Preferred volume 1 mL serum
Min. Volume 0.5 mL serum
Processing notes Spin and freeze aliquot at -20C. Ship to China Basin.
Ref Lab Rejection Criteria Gross hemolysis, lipemia or icterus
Units U
Normal range DESMOGLEIN 1
Negative <14.0 U
Indeterminate 14.0-20.0 U
Positive >20.0 U

Negative <9.0 U
Indeterminate 9.0-20.0 U
Positive >20.0 U
Synonyms DSG1 Ab; DSG1 Antibody; DSG3 Ab; DSG3 Antibody
Stability Room temperature 1.5 days, refrigerated 1 week, frozen 2 weeks
Turn around times 7-9 days
Additional 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 (ELISA) methods.

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.
CPT coding 83516-90 x2
Last Updated 5/22/2013 1:28:58 PM
Entry Number 1469
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