023
Test #
Collagen VII Antibody ELISA
CPT Code(s) #
83520
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.
Specimen Requirements:
Collect 2-5 ml of blood in a red top Vacutainer or serum separator (SST) tube, allow blood to clot, and centrifuge. Label tube appropriately.
Sample Stability:
Room temperature = stable during shipment
2ºC to 8ºC = 5 days
-25ºC to -15ºC = 1 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).
