Desmoglein 1 (DSG1) antibody

Category: Vesiculo-Bullous Disease
Test Code: 112
Disease: Pemphigus/Pemphigoid/EBA
Methodology: ELISA
Reference Range: Negative: <14.0 ; Indeterminate: 14.0-20.0 ; Positive; >20.0
Units: U/ml
CPT Code: 83516 (x1)
Schedule / Turnaround Time: Assay performed once every two weeks. Report availability is two weeks from the time of specimen receipt.
Specimen Requirements:

Specimen need not be refrigerated or frozen. Collect 2-3 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into white tube provided with Immco Diagnostics’ collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.

Sample Stability:

Sample is stable at ambient temperature during shipment. If sample is stored prior to shipment, it is stable refrigerated (2-8°C) up to five days and frozen (-20°C or lower) up to one year.

Relevance:

Pemphigus includes a group of often fatal, autoimmune blistering diseases characterized by intraepithelial lesions. Pemphigus vulgaris and its variants may present with oral or other mucosal lesions alone or with mucosal plus skin lesions. Pemphigus foliaceus and its variants present with skin lesions alone. Indirect Immunofluorescence studies reveal that both forms of pemphigus are caused by autoantibodies to cell surface antigens of stratified epithelia of mucous membranes and skin. These antibodies bind to calcium dependent adhesion molecules in cell surface desmosomes, notably desmoglein 1(DSG-1) in pemphigus foliaceus and desmoglein 3 (DSG-3) in pemphigus vulgaris. Pemphigus vulgaris patients with both mucosal and skin lesions have antibodies to both DSG-3 and DSG-1. The diagnosis of pemphigus depends on biopsy and serum studies that characterize lesions and detect the autoantibodies that cause them. Serum studies afford highly sensitive diagnostic aids. Originally they were performed by indirect immunofluorescence using monkey esophagus and other tissues sections. The identification of the reactive antigens as DSG-1 and DSG-3 has made it possible to develop highly specific and sensitive ELISA methods.