Test Catalog

Test Id : PNPAB

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Paraneoplastic Pemphigus Antibody, IgG, Serum

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Overview

Test Catalog

Useful For
Suggests clinical disorders or settings where the test may be helpful

Diagnosis of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome in the setting of erosive or lichenoid mucocutaneous disease

Method Name
A short description of the method used to perform the test

Indirect Immunofluorescence Assay (IFA)

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

Paraneoplastic Pemphigus, IgG Ab, S

Aliases
Lists additional common names for a test, as an aid in searching

Basement Membrane Antibodies

Bullous Lupus Erythematosus

Bullous Pemphigoid

Cicatricial Pemphigoid

Circulating IgG Basement Membrane Zone Antibody

Circulating Intercellular Ab Study

Epidermal Cell Surface Antibody

Epidermal Fluorescence Antibody

Epidermolysis Bullosa Acquisita

Epithelial Antibody

Herpes gestationis

IMF - Epidermal Fluorescent Ab

Indirect Immunofluorescence

Intercellular Substance Antibody

Lupus Erythematosus (LE)

Paraneoplastic Pemphigus (PNP)

Pemphigoid

Pemphigus

Skin Basement Membrane Antibodies

Split-Skin Assay

PAMS- Paraneoplastic autoimmune multiorgan syndrome

Specimen Type
Describes the specimen type validated for testing

Serum

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Collection Container/Tube:

Preferred: Serum gel

Acceptable: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 2 mL

Collection Instructions: Centrifuge and aliquot serum into a plastic vial.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

0.5 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus Reject

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 14 days
Frozen 30 days
Ambient 14 days

Useful For
Suggests clinical disorders or settings where the test may be helpful

Diagnosis of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome in the setting of erosive or lichenoid mucocutaneous disease

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Paraneoplastic pemphigus (PNP) (also paraneoplastic autoimmune multiorgan syndrome [PAMS] to denote the systemic nature of the syndrome) is an autoimmune mucocutaneous blistering disease affecting adults or, rarely, children that generally heralds the presence of an underlying malignancy.

 

PNP/PAMS can be defined and identified by a combination of the following features:

1. Painful stomatitis and a polymorphous cutaneous eruption with lesions that may be blistering, lichenoid, erythema multiforme-like, or morbilliform

2. Variable histopathologic findings, including acantholysis, lichenoid, or interface change

3. Variable direct immunofluorescence findings from a perilesional biopsy, often demonstrating deposition of IgG and complement in the epidermal intercellular spaces, granular/linear complement deposition along the epidermal basement membrane zone, or a lichenoid tissue reaction

4. Indirect immunofluorescence evidence of cell surface deposition on primate esophagus or rat bladder epithelium

5. Enzyme-linked immunosorbent assay evidence of serum autoantibodies against desmogleins 1 or 3 and, possibly, against bullous pemphigoid 180 and 230 antigens

The incidence of the disease is unknown, but it is less common than pemphigus vulgaris or foliaceus. Clinical features of the disease can mimic those seen in a drug reaction, erythema multiforme, Stevens-Johnson syndrome, pemphigus, lichen planus, or toxic epidermal necrolysis.

 

In the majority of cases, PNP/PAMS is associated with non-Hodgkin lymphoma, chronic lymphocytic leukemia, thymoma, or Castleman disease. A serious complication includes bronchiolitis obliterans, which may lead to respiratory failure.

Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

Negative

Interpretation
Provides information to assist in interpretation of the test results

In the appropriate clinical setting, a positive result can support a diagnosis of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS). However, correlation with clinical features, histopathologic findings, results of serum studies (such as indirect immunofluorescence on primate esophagus substrate and enzyme-linked immunosorbent assay for Dsg1/3) is required for a final diagnosis.

 

As the test is not entirely sensitive, a negative test result does not exclude the possibility of PNP/PAMS.

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

Test results must be interpreted in the patient's individual clinical context.

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med. 1990;323(25):1729-1735. doi:10.1056/NEJM199012203232503

2. Anhalt GJ, Aris-Abdo L, Bonitz P, Labib RS. Antigen specificity of paraneoplastic pemphigus: predictive value of diagnostic techniques based on the study of 17 patients and 135 control subjects. J Invest Dermatol. 1992(4);98:580. Abstract 172

3. Liu AY, Valenzuela R, Helm TN, Camisa C, Melton AL, Bergfeld WF. Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. J Am Acad Dermatol. 1993;28(5 Pt 1):696-699. doi: 10.1016/0190-9622(93)70095-b

4. Camisa C, Helm TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol. 1993;129(7):883-886

5. Montagnon CM, Tolkachjov SN, Murrell DF, et al. Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol. 2021;84(6): 1507-1519. doi:10.1016/j.jaad.2020.11.075

6. Montagnon CM, Lehman JS, Murrell DF, et al. Intraepithelial autoimmune bullous dermatoses: disease activity assessment and therapy. J Am Acad Dermatol. 2021;84(6);1523-1537. doi:10.1016/j.jaad.2021.02.073

Method Description
Describes how the test is performed and provides a method-specific reference

Commercially-prepared sections of rat bladder (substrate) are overlaid with patient’s serum; incubated, covered with fluorescein-conjugated IgG antiserum, and interpreted using fluorescent microscopy.(Unpublished Mayo method)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Wednesday through Friday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

7 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

30 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

  • Authorized users can sign in to Test Prices for detailed fee information.
  • Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
  • Prospective clients should contact their account representative. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

86255

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
PNPAB Paraneoplastic Pemphigus, IgG Ab, S 93233-5
Result Id Test Result Name Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
61881 Paraneoplastic Pemphigus, IgG Ab, S 93233-5

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | PHP Pdf | CMS Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports