Ordering Recommendation

Use in conjunction with Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF (0092107) to aid in the diagnosis of paraneoplastic pemphigus. May be used to monitor disease in individuals with increased IgG envoplakin antibody levels. 

Panel testing that includes IgG antibodies by indirect immunofluorescence is preferred; refer to Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA (3016534).

Results should be correlated with Direct Immunofluorescence, Tissue Biopsy (Cutaneous, Mucosal, Epithelial) (0092572); histopathological examination of formalin-fixed tissue; and assessment of other epithelial antibodies.

For comprehensive testing, order with: 
•    Immunobullous Disease Antibody Panel (3001409). 

Alternatively, to assess for specific disease-associated antibodies or individual antibody targets, order with: 
•    Pemphigus Antibody Panel, IgG (0090650),
•    Pemphigus Antibodies, IgA by IIF (0092106), and
•    Basement Membrane Zone Antibody Panel (3001410)
Or
•    Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA (0090649),
•    Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA (0092566), and
•    Collagen Type VII Antibody, IgG by ELISA (2010905).

For more information about components, methodology, and specimen type, refer to the ARUP Immunobullous Disease Testing Comparison tool.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation
Collect

Plain red or serum separator tube (SST).

Specimen Preparation

Transfer 2 mL serum to an ARUP standard transport tube. (Min: 0.5 mL)

Storage/Transport Temperature

Refrigerated.

Unacceptable Conditions

Hemolyzed or lipemic specimens. Plasma

Remarks
Stability

Ambient: 1 week; Refrigerated: 2 weeks; Frozen: Indefinitely

Methodology

Semi-Quantitative Enzyme-Linked Immunosorbent Assay (ELISA)

Performed

Varies

Reported

7-14 days

Reference Interval

Interpretive Data

Refer to report.

Compliance Category

Performed by non-ARUP Laboratory

Note

The methodology is enzyme-linked immunosorbent assay (ELISA) to detect IgG antibodies to envoplakin.

This test should be distinguished from antibody testing of cerebral spinal fluid (CSF) for paraneoplastic neurologic syndromes; 3004510, 3004512, 3004517 are different tests.

Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare paraneoplastic disease that affects patients of all ages, is associated with lymphoproliferative disorders/malignancies and demonstrates clinical features of severe pemphigus with a high mortality rate.

Patients with PNP/PAMS develop serum antibodies to multiple epithelia (simple, columnar, transitional) with several possible epithelial antigen targets. Envoplakin is one of several possible epithelial targets, albeit a major one. The IgG envoplakin antibody level by ELISA, increased or normal (positive or negative), as a stand-alone diagnostic test, does not completely confirm or rule out a diagnosis of PNP/PAMS; its usefulness is as a marker in conjunction with other indicators. Additionally, IgG envoplakin antibody levels, when increased, may correlate with disease extent and activity and, therefore, can be useful in disease monitoring.

Hotline History

N/A

CPT Codes

83516

Components

Component Test Code* Component Chart Name LOINC
3016535 Envoplakin Antibody, IgG by ELISA
3016536 EER Envoplakin Antibody, IgG by ELISA 11502-2
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.

Aliases

  • Aliases envoplakin antibodies
  • mixed epithelial antibodies
  • paraneoplastic autoimmune multiorgan syndrome (PAMS)
  • Paraneoplastic Pemphigus Antibody Screen
  • periplakin antibodies
  • rat bladder immunofluorescence
Envoplakin Antibody, IgG by ELISA