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Paraneoplastic pemphigus (paraneoplastic autoimmune multi-organ syndrome) is a severely debilitating blistering disease affecting skin and mucous membranes in patients with malignancy, particularly hematologic malignancies.
Tests generally appear in the order most useful for common clinical situations
| Test name: Paraneoplastic Pemphigus Antibody Screen |
| ARUP #: 0092107 |
| Methodology: Indirect Fluorescent Antibody |
| Use: Indirect immunofluorescence testing of serum for paraneoplastic pemphigus IgG antibodies in patient suspected of having a blistering disease associated with malignancy Uses substrates from rodents, including mouse heart, liver, bladder and rat bladder to detect characteristic staining Consider concurrent pemphigus panel test to detect and monitor pemphigus antibodies, if present (including IgG desmoglein 1 and desmoglein 3 antibodies) and concurrent pemphigoid panel test to detect and monitor basement membrane zone (BMZ) antibodies |
| Limitations: Clinical correlation is necessary because results may overlap with other types of pemphigus and with pemphigoid Rarely, patients may also have IgA paraneoplastic pemphigus antibodies; IgA paraneoplastic pemphigus testing can be performed by request |
| Follow-up: Monitor paraneoplastic pemphigus antibodies and/or pemphigus panel, particularly if IgG desmoglein 1 or 3 antibodies are increased |
| Test name: Cutaneous Direct Immunofluorescence, Biopsy |
| ARUP #: 0092572 |
| Methodology: Direct Immunofluorescence (Direct Fluorescent Antibody Stain) |
| Use: Determine presence and staining pattern of immunoglobulins (IgG, IgM, IgA), third component of complement (C3) and fibrinogen in perilesional skin or mucosal biopsy specimens from patients suspected of having pemphigus; perform this test with serum pemphigus panel |
| Limitations: May be inaccurate if tissue not taken from correct perilesional location (attached/intact epithelium or epidermis is needed) Not possible to reliably distinguish pemphigus subtypes based on direct immunofluorescence (DIF); serum testing helpful for subtyping Tissue must be submitted in Michel’s or Zeus medium; this test cannot be performed on formalin-fixed tissue |
| Follow-up: Concurrent serum testing with pemphigoid panel is the most sensitive for diagnosis and for determining subtype of subepidermal blistering disease Patients with indeterminate results should have repeat biopsy for direct immunofluorescence and antibody levels monitored for disease activity |
| Test name: Pemphigus Panel - IgG Epithelial Cell Surface Antibodies and Levels of IgG Desmoglein 1 and Desmoglein 3 Antibodies, Serum |
| ARUP #: 0090650 |
| Methodology: Enzyme-Linked Immunosorbent Assay/Indirect Fluorescent Antibody |
| Comments: IgG cell surface antibody staining is found in all cases; IgG desmoglein 1 and/or IgG desmoglein 3 antibody levels may be increased |
| Test name: Pemphigoid Panel - Epithelial Basement Membrane Zone IgG & IgA, BP180 & BP230 IgG Antibodies |
| ARUP #: 0092001 |
| Methodology: Enzyme-Linked Immunosorbent Assay/Indirect Fluorescent Antibody |
| Comments: IgG BMZ antibody staining is found in some cases; IgG BP180 and IgG BP230 antibody levels are typically in normal range |