Ordering Recommendation

Preferred expanded panel to aid in diagnosis and monitoring of paraneoplastic pemphigus. Testing includes IgG envoplakin antibodies by ELISA and IgG antibodies by indirect immunofluorescence using rat bladder and other rodent substrates. 

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
•    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

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


Unacceptable Conditions

Hemolyzed or lipemic specimens. Plasma.


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


Semi-Quantitative Indirect Immunofluorescence (IIF)/Semi-Quantitative Enzyme-Linked Immunosorbent Assay (ELISA)




7-14 days

Reference Interval

By report

Interpretive Data

Refer to report

Compliance Category

Performed by non-ARUP Laboratory


The methodology is indirect immunofluorescence (IIF) of patient serum with rodent substrates including rat bladder, mouse bladder, mouse heart, and mouse liver to detect characteristic IgG antibody reactivity: simple columnar epithelial cell surface and basement membrane zone in bladders, intercalated discs in heart, and portal tracts in liver, and enzyme-linked immunosorbent assay (ELISA) to detect IgG antibodies to envoplakin. Monkey esophagus substrate is included if other concurrent IIF testing does not.

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, and IgG envoplakin antibody levels correlate with extent of mucocutaneous disease in patients with PNP/PAMS.

Hotline History


CPT Codes

88346; 88350 x3; 83516


Component Test Code* Component Chart Name LOINC
3016537 Paraneoplastic Pemphigus Expanded Ab
3016538 EER Paraneoplastic Pemphigus Expanded Ab 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 cell surface and basement membrane zone antibodies
  • envoplakin antibodies
  • intercellular substance antibodies
  • mixed epithelial antibodies
  • paraneoplastic autoimmune multiorgan syndrome (PAMS)
  • paraneoplastic pemphigus (PNP)
  • periplakin antibodies
  • plakin antibodies
  • rat bladder immunofluorescence
Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA