Test Code #

015

CPT Code(s) #

83516 (x2)

Test Name

Desmoglein (Dsg) 1 & Dsg 3 Abs

If Profile, Includes Tests:

N/A

Disease Name:

Pemphigus, Paraneoplastic Pemphigus

Type of Study:

Serum Studies

Methodology:

ELISA

Substrate:

Reference Range:

Dsg1
Negative: <18 U/ml
Indeterminate 18-36 U/ml
Positive > 36 U/ml
Dsg3
Negative: <19 U/ml
Indeterminate: 19-37 U/ml
Positive: > 37 U/ml

Units:

U/ml

Schedule:

Assay performed once every week. Report availability is within one week from the time of specimen receipt

Specimen Requirements:

Collect 5-10 ml of blood in a red top or serum separator tube. If possible, separate serum from clot and place into red capped tube provided with Beutner Laboratories collection kits. If separation facilities are not available, the blood can be sent in the tube used for collection.

Sample Stability:

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

Clinical Relevance:

Pemphigus includes a group of often fatal autoimmune blistering diseases characterized by intraepidermal and/or 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 epidermal layer of the 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.

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