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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, Serum

Specimen Type

Serum

Specimen 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 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test 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