023
Test #
Collagen VII Antibody ELISA
CPT Code(s) #
83516
If Profile, Includes Tests:
N/A
Disease Name:
Epidermolysis Bullosa Acquisita (EBA), Bullous LE
Type of Study:
Serum Studies
Methodology:
ELISA
Substrate:
N/A
Reference Range:
< 6.0 U/ml Negative
≥ 6.0 U/ml Positive
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:
Type VII Collagen is found in the lamina densa and the sublamina densa fibrillar area of the dermal-epidermal junction. The pathogenicity of autoantibodies targeting collagen VII has been independently demonstrated both in vitro, ex vivo and in vivo. Collagen VII autoantibodies specific to COL7 is the current standard for EBA diagnosis(Hashimoto, 2012). Type VII collagen (COL7)-specific autoantibodies were primarily of the IgG isotype and were seen in over 75% of the EBA patients (Iwata, 2018). The immunodominant domains of type VII collagen can also be recognized by IgG autoantibodies from patients with bullous lupus erythematosus (BSLE). Antitype VII collagen autoantibodies can be detected 3 months before the inception of BSLE in patients with SLE. In a study by Grabell (2015) of BSLE, 100% of the patient had circulating Collagen VII antibodies before and during the skin eruption. The healthy and LSE controls did not show any circulating collagen VII antibodies (Grabell, 2015, Ishikawa,1997).
