Test Code DSGAB Desmoglein 1 (DSG1) and Desmoglein 3 (DSG3), IgG Antibodies, Serum
Specimen Required
Collection Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Submission Container/Tube: Plastic vial
Specimen Volume: 1 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
Useful For
Preferred screening test for patients suspected to have an autoimmune blistering disorder of the skin or mucous membranes (pemphigus)
Aiding in the diagnosis of pemphigus
Method Name
Enzyme-Linked Immunosorbent Assay (ELISA)
Reporting Name
Desmoglein 1 and 3, SerumSpecimen Type
SerumSpecimen Minimum Volume
0.5 mL
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 14 days | |
Frozen | 30 days | ||
Ambient | 14 days |
Reference Values
DESMOGLEIN 1:
<20 RU/mL (negative)
≥20 RU/mL (positive)
DESMOGLEIN 3:
<20 RU/mL (negative)
≥20 RU/mL (positive)
Interpretation
Antibodies to desmoglein 1 (DSG1) and desmoglein 3 (DSG3) have been shown to be present in patients with pemphigus. Many patients with pemphigus foliaceus, a superficial form of pemphigus have antibodies to DSG1. Patients with pemphigus vulgaris, a deeper form of pemphigus, have antibodies to DSG3 and sometimes DSG1 as well.
Antibody titer correlates in a semiquantitative manner with disease activity in many patients. Patients with severe disease can usually be expected to have high titers of antibodies to DSG. Titers are expected to decrease with clinical improvement.
Our experience demonstrates a very good correlation between DSG1 and DSG3 results and the presence of pemphigus. Adequate sensitivities and specificity for disease are documented. However, in those patients strongly suspected to have pemphigus either by clinical findings or by routine biopsy, and in whom the DSG assay is negative, indirect immunofluorescence testing is recommended. For more information see CIFS / Cutaneous Immunofluorescence Antibodies (IgG), Serum.
Clinical Reference
1. Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999;40(2 Pt 1):167-170
2. Amagai M, Komai A, Hashimoto T, et al. Usefulness of enzyme-linked immunoabsorbent assay using recombinant desmogleins 1 and 3 for sero-diagnosis of pemphigus. Brit J Dermatol. 1999;140(2):351-357
3. Harman KE, Gratian MJ, Bhogal BS, Challacombe SJ, Black M. The clinical significance of autoantibodies to desmoglein 1 in 78 cases of pemphigus vulgaris. J Invest Derm. 1999;112(4):568. Abstract 273
4. Harman KE, Gratian MJ, Seed PT, Bhogal BS, Challacombe SJ, Black MM. Diagnosis of pemphigus by ELISA: a critical evaluation of two ELISAs for the detection of antibodies to the major pemphigus antigens, desmoglein 1 and 3. Clin Exp Dermatol. 2000;25(3):236-240
5. Prussmann W, Prussmann J, Koga H, et al. Prevalence of pemphigus and pemphigoid autoantibodies in the general population. Orphanet J Rare Dis. 2015;10:63
6. Toosi S, Collins JW, Lohse CM, et al. Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus. Int J Dermatol. 2016;55(4):e184-e190
7. Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS. Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol. 2021;84(6):1507-1519
Day(s) Performed
Varies
Report Available
1 to 10 daysPerforming Laboratory
Mayo Clinic Laboratories in RochesterTest Classification
This test has been modified from the manufacturer's instructions. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
83516 x 2
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
DSGAB | Desmoglein 1 and 3, Serum | 94335-7 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
606818 | DSG 1 | 94336-5 |
606819 | DSG 3 | 94337-3 |