Ordering Recommendation

Use to assess and monitor paraneoplastic pemphigus, a rare paraneoplastic disease associated with lymphoproliferative disorders/malignancies and demonstrating clinical features of severe pemphigus. Testing should be correlated with concurrent Direct Immunofluorescence, Tissue Biopsy (Cutaneous, Mucosal, Epithelial) (0092572), histopathological examination of formalin-fixed tissue, and assessment of other epithelial antibodies. 

Consider ordering concurrently with Immunobullous Disease Antibody Panel (3001409) for broad epithelial antibody assessment. Alternatively, order with Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA (0090649) and Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA (0092566) because any of these epithelial antibodies may be increased in paraneoplastic pemphigus.

If other more common types of pemphigus are diagnostic considerations, order Pemphigus Antibody Panel, IgG (0090650) with or without the rarer IgA pemphigus variant test, Pemphigus Antibodies, IgA by IIF (0092106), concurrently with this test.

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




Semi-Quantitative Indirect Immunofluorescence (IIF)




3-7 days

New York DOH Approval Status

This test is New York DOH 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

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 on substrates from rodents including rat bladder, mouse bladder, mouse heart, and mouse liver to detect characteristic antibody reactivity: simple columnar epithelial cell surface and basement membrane zone in bladders, intercalated discs in heart, and portal tracts in liver. Monkey esophagus substrate is included if other concurrent IIF testing does not. For specimens less than 0.5 mL, call the Immunodermatology Laboratory at (866) 266-5699.

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

Hotline History


CPT Codes

88346; 88350 x4


Component Test Code* Component Chart Name LOINC
0092107 Paraneoplastic Pemphigus Ab Screen 42936-5
* 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.


  • cell surface and basement membrane zone antibodies
  • envoplakin antibodies
  • intercellular substance
  • mixed epithelial antibodies
  • paraneoplastic autoimmune multiorgan syndrome (PAMS)
  • Paraneoplastic Pemphigus Antibody Screen
  • periplakin antibodies
  • PNP
  • rat bladder immunofluorescence
Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Antibody Screening Panel