Bullous autoimmune dermatoses belong to organ - specific autoimmune diseases. They are characterized by formation of autoantibodies against structural proteins of the skin. In Pemphigus disease the blisters are formed intra epidermally whereas in Bullous autoimmune dermatoses they occur sub epidermally. Thus this assay helps in further differentiation of blistering diseases of the skin. Desmoglein 3 (Dsg3), also known as Cadherin family member 6 (CDHF6), is a member of the desmosomal cadherin family and plays a critical role in cell-cell adhesion. It is predominantly expressed in stratified squamous epithelium, including the skin, oral mucosa, and esophagus. Desmoglein 3 has been characterized as the autoantigen of pemphigus vulgaris (PV), a life-threatening autoimmune disease. Binding of anti-Desmoglein 3 autoantibodies causes the loss of cell-cell adhesion, which clinically manifests as blisters on the skin and oral mucosa, and histologically as suprabasilar acantholysis.
Specimen should be collected in fasting or at least one hour after meals to avoid lipemia. 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.
Preferred screening test for patients suspected to have an autoimmune blistering disorder of the skin or mucous membranes (pemphigus). Monitor disease in patient previously diagnosed with pemphigus and increased IgG desmoglein 1; antibody levels correlate with disease activity. If used to screen for pemphigus, this test has decreased sensitivity and specificity when compared to Pemphigus Antibody Panel - Epithelial Cell Surface Antibodies and Desmoglein 1. For initial diagnosis and monitoring disease, Pemphigus Antibody Panel - Epithelial Cell Surface Antibodies and Desmoglein 1 is preferred.
Aseptically collect 5-8 mL of blood by approved venepuncture procedures. Allow the blood to clot at room temperature (20-25° C) and separate as soon as possible to limit haemolysis. Anticoagulants or preservatives should be avoided. Avoid using sera exhibiting a high level of lipemia, haemolysis, icterus or microbial growth. These conditions may cause aberrant test results. Do not use heat-inactivated serum as this affects test results.Blood should be collected fasting or at least one hour after meals to avoid lipemic serum.
Type | Gender | Age-Group | Value |
---|---|---|---|
DESMOGLEIN 3
|
UNISEX
|
All age groups
|
<9.0 U (negative),9.0-20.0 U (indeterminate),>20.0 U (positive)
|