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Pemphigoid is a chronic autoimmune blistering disease of the skin and mucous membranes.
Tests generally appear in the order most useful for common clinical situations
| Test name: Cutaneous Direct Immunofluorescence, Biopsy |
| ARUP #: 0092572 |
| Methodology: Direct Immunofluorescence (Direct Fluorescent Antibody Stain) |
| Use: Use to determine the presence and characteristic staining pattern of immunoglobulins (IgG, IgM, IgA), third component (C3) of complement and fibrinogen in skin or mucous membrane biopsy specimens (biopsy site is critical; see below) from patients suspected of having pemphigoid; perform this test with serum pemphigoid panel For skin involvement, biopsy perilesional skin For mucous membrane involvement, biopsy nonlesional mucosa See Immunobullous Skin Diseases Testing algorithm |
| Limitations: May be inaccurate if tissue not taken from correct perilesional location (attached/intact epithelium or epidermis is needed) Not possible to distinguish pemphigoid from epidermolysis bullosa acquisita by direct immunofluorescence (DIF); concurrent serum testing needed Tissue must be submitted in Michel’s or Zeus medium; this test cannot be performed on formalin-fixed tissue |
| Follow-up: Initial concurrent and repeat serum testing with pemphigoid panel is the most sensitive for diagnosis, for distinguishing pemphigoid from other subepithelial immunobullous disease, and for following disease activity Patients with indeterminate results should have repeat DIF biopsy Patients with changing clinical features should have repeat DIF biopsy because antibody profiles may change over time See Immunobullous Skin Diseases Testing algorithm |
| Test name: Pemphigoid Panel - Epithelial Basement Membrane Zone IgG & IgA, BP180 & BP230 IgG Antibodies |
| ARUP #: 0092001 |
| Methodology: Enzyme-Linked Immunosorbent Assay/Indirect Fluorescent Antibody |
| Use: Panel includes epithelial basement membrane zone (BMZ) IgG & IgA antibodies by indirect immunofluorescence (IFA) on split human skin and monkey esophagus substrates, BP180 & BP230 IgG antibodies by ELISAs Use to diagnose most types of pemphigoid, epidermolysis bullosa acquisita, linear IgA disease (including linear IgA bullous dermatosis and chronic bullous disease of childhood), mixed immunobullous disease Use along with pemphigus panel and endomysial antibody IgA testing to initially diagnose and discriminate among the immunobullous skin diseases in patients suspected or known to have any type of immunobullous disease Use to monitor disease activity and therapeutic response Concurrent perilesional skin biopsy for DIF is important for diagnosis because of increased sensitivity (85-100% of pemphigoid cases are positive) See Immunobullous Skin Diseases Testing algorithm |
| Limitations: Clinical correlation necessary because the incidence of false positives, although rare, increases with age Because of clinical overlap among immunobullous diseases and similar names, pemphigoid testing may be confused with pemphigus testing and inadvertently misordered |
| Follow-up: Use pemphigoid panel or Bullous Pemphigoid (180 kDa and 230 kDa) Antigens, IgG to monitor pemphigoid disease activity and response to therapy Repeat pemphigoid panel for indeterminate results and/or continuing clinical consideration of the disease See Immunobullous Skin Diseases Testing algorithm |
| 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 |
| Use: Panel includes epithelial cell surface IgG antibodies by IFA on intact human skin and monkey esophagus substrates, IgG desmoglein 1 and IgG desmoglein 3 antibodies by ELISAs Use to diagnose most major types of pemphigus and to monitor disease activity and therapeutic response Use along with pemphigoid panel and endomysial IgA antibody tests to initially diagnose and distinguish various immunobullous disorders in patients suspected or known to have any type of immunobullous disease See Immunobullous Skin Diseases Testing algorithm |
| Limitations: Clinical correlation is necessary because cell surface antibodies by IFA, usually in low titers, may be found in normal individuals (possible blood group reactivity) or in patients with fungal infections, burns, drug reactions, and other dermatoses, including other immunobullous diseases Because of clinical overlap among immunobullous diseases and similar names, pemphigoid testing may be confused with pemphigus testing and inadvertently misordered Testing for IgG pemphigus antibody types (most common) also may be confused with IgA pemphigus testing (rare disorder) |
| Test name: Tissue Transglutaminase (tTG) Antibody, IgA with Reflex to Endomysial Antibody, IgA by IFA |
| ARUP #: 0050734 |
| Methodology: Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody |
| Use: Use along with pemphigoid and pemphigus panel tests, or use with epithelial skin antibody testing to initially diagnose and discriminate among the immunobullous skin diseases in patients suspected or known to have any type of immunobullous disease |
| Limitations: Does not detect IgG or IgA BMZ antibodies that characterize subepidermal immunobullous diseases other than dermatitis herpetiformis |
| Test name: Epithelial Skin Antibody |
| ARUP #: 0090299 |
| Methodology: Indirect Immunofluorescence (Indirect Fluorescent Antibody) |
| Use: Panel includes epithelial BMZ IgG and IgA antibodies by IFA and IgG and IgA cell surface antibodies by IFA on split human skin, intact human skin, and monkey esophagus substrates Use as alternate to pemphigoid and pemphigus panel tests to initially diagnose and discriminate among clinically similar immune-mediated skin diseases such as pemphigoid, linear IgA disease, epidermolysis bullosa acquisita, pemphigus, and dermatitis herpetiformis in patients suspected of having or known to have any type of subepidermal immunobullous disease |
| Limitations: Does not include testing for antibodies to target pemphigoid antigens, BP180 and BP230; which may be more sensitive diagnostic markers in some cases and the levels correlate with disease activity Although helpful in screening for immunobullous disease, test is not as sensitive as combination of pemphigus and pemphigoid panels |
| Follow-up: Use epithelial skin antibody test or both pemphigoid and pemphigus panels to follow patients with changing clinical features because antibody profiles may change over time |
| Test name: Epithelial Basement Membrane Zone IgG Antibodies |
| ARUP #: 0092056 |
| Methodology: Indirect Immunofluorescence (Indirect Fluorescent Antibody) |
| Use: This test comprises components included in pemphigoid panel, epithelial skin antibody, and pemphigoid gestationis tests Use to establish or confirm diagnosis of pemphigoid or epidermolysis bullosa acquisita in patients suspected of having or known to have any type of disorder with IgG BMZ antibodies Use to distinguish pemphigoid from epidermolysis bullosa acquisita |
| Limitations: Clinical correlation necessary because the incidence of false positives, although rare, increases with age This test does NOT detect IgA BMZ antibodies; linear IgA disease and dermatitis herpetiformis will NOT be detected IgG BP180 and BP230 antibody levels, which may increase sensitivity in diagnosing pemphigoid and are useful to establish baseline for monitoring disease activity and response to therapy, are NOT included in this test |
| Follow-up: Use epithelial basement membrane zone IgG antibodies test to follow patients with pemphigoid, particularly those with normal bullous pemphigoid (180 kDa and 230 kDa) antigens IgG antibody tests Repeat epithelial basement membrane zone IgG antibodies test for indeterminate results and/or continuing clinical consideration of pemphigoid See Immunobullous Skin Diseases Testing algorithm |
| Test name: Bullous Pemphigoid (180 kDa and 230 kDa) Antigens, IgG |
| ARUP #: 0092566 |
| Methodology: Enzyme-Linked Immunosorbent Assay |
| Use: This test comprises components included in the pemphigoid panel and pemphigoid gestationis factor test Use to test for IgG BP180 (BP Ag2) and BP230 (BP Ag1) antibodies in patients suspected of having or known to have any type of pemphigoid, including bullous pemphigoid and variant forms, mucous membrane (cicatricial) pemphigoid, and herpes gestationis Use to measure relative levels of BP180 and BP230 IgG antibodies to support a diagnosis of pemphigoid and to monitor disease activity and therapeutic response Use along with epithelial BMZ antibodies by IFA to identify patients with pemphigoid antibodies to epitopes other than those in the assay |
| Limitations: Negative, positive, and borderline/indeterminate results should be correlated and confirmed with IFA, either epithelial skin antibody test or epithelial BMZ, IgG test and indeterminate/borderline levels should be monitored Patients with pemphigoid may show reactivity to multiple BMZ components; therefore, negative/normal IgG BP180 and BP230 antibody levels do not rule out pemphigoid or another subepidermal immunobullous disease Up to 7% of individuals unaffected by pemphigoid (including those with other immunobullous diseases) have increased IgG BP 180 and/or IgG BP 230 antibody levels These tests are components of pemphigoid panel; pemphigoid panel is optimal for initial diagnosis of pemphigoid and monitoring response to therapy; otherwise, concurrent testing is advised with one of the following
Correlations with IFA (epithelial antibody test), DIF findings, and clinical presentation are important for initial diagnosis |
| Follow-up: Use bullous pemphigoid (180 kDa and 230 kDa) antigens IgG antibody tests to follow disease activity in pemphigoid Patients with pemphigoid may show antibody reactivity that changes over time; therefore, testing by both IFA for BMZ antibodies and BP 180 and BP 230 ELISAs with the pemphigoid panel is strongly recommended periodically to follow disease activity See Immunobullous Skin Diseases Testing algorithm |