Back
Menu
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: Determine presence and staining pattern of immunoglobulins (IgG, IgM, IgA), third component of complement 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 by DIF and antibody levels monitored for disease activity |
| 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: Diagnose most types of pemphigoid, epidermolysis bullosa acquisita, linear IgA disease (including linear IgA bullous dermatosis and childhood immunobullous disease) 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 ELISA Use in conjunction with pemphigus panel and endomysial antibody IgA testing to differentiate among the immunobullous skin diseases in patients suspected or known to have any type of immunobullous disease IgG BP180 and BP230 antibody levels may be useful in monitoring disease activity and response to therapy |
| Limitations: Clinical correlation necessary since incidence of false positives, although rare, increases with age Because of clinical overlap among immunobullous diseases and similar names, testing for pemphigoid may be confused with testing for pemphigus and misordered |
| Follow-up: Perilesional skin biopsy by DIF is helpful in diagnosis (>90% of pemphigoid and epidermolysis bullosa acquisita cases are positive) Use pemphigoid panel to monitor pemphigoid disease activity and response to therapy |
| 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: Diagnose most types of pemphigus and monitor disease activity and response to therapy Panel tests for IgG epithelial cell surface antibodies by IFA on intact human skin and monkey esophagus substrates and IgG desmoglein 1 and IgG desmoglein 3 antibodies by ELISA Use along with pemphigoid panel and endomysial IgA antibody testing, or epithelial skin antibody testing to distinguish the various disorders in patients suspected or known to have any type of immunobullous disease |
| 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 Because of clinical overlap among immunobullous diseases and similar names, testing for pemphigus may be confused with testing for pemphigoid and misordered; testing for IgG pemphigus antibody types (most common) also may be confused with IgA pemphigus testing (rare disorder) |
| Follow-up: Concurrent perilesional skin biopsy evaluated by DIF is important for diagnosis because of increased sensitivity (85-100% of pemphigus, pemphigoid, linear IgA disease, epidermolysis bullosa acquisita, and dermatitis herpetiformis cases are positive) Use pemphigus panel to monitor disease activity and response to therapy |
| 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 epithelial skin antibody testing to differentiate among the immunobullous skin diseases in patients suspected or known to have any type of immunobullous disease |
| Follow-up: Perilesional skin biopsy by DIF is helpful in diagnosis (>90% of dermatitis herpetiformis cases are positive) |
| Test name: Epithelial Skin Antibody |
| ARUP #: 0090299 |
| Methodology: Indirect Immunofluorescence (Indirect Fluorescent Antibody) |
| Use: Discriminate among clinically similar immune-mediated skin diseases such as linear IgA disease, pemphigoid, epidermolysis bullosa acquisita and dermatitis herpetiformis in patients suspected of having or known to have any type of subepidermal immunobullous disease Panel includes epithelial BMZ IgG & IgA antibodies by IFA and IgG & IgA cell surface antibodies by IFA on split human skin, intact human skin and monkey esophagus substrates |
| Limitations: Although helpful in screening for immunobullous disease, not as sensitive as combination of pemphigus and pemphigoid panels |
| Follow-up: Perilesional skin biopsy by DIF is helpful in diagnosis (>90% of pemphigus, pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, dermatitis herpetiformis cases are positive) IgG desmoglein 1 and 3 antibodies and/or IgG BP180 and BP230 antibodies tests are helpful in determining immunobullous disease subtype and monitoring response to therapy |
| Test name: Epithelial Basement Membrane Zone IgG Antibodies |
| ARUP #: 0092056 |
| Methodology: Indirect Immunofluorescence (Indirect Fluorescent Antibody) |
| Use: Establish diagnosis of pemphigoid or epidermolysis bullosa acquisita in patients suspected of having or known to have any type of pemphigoid or epidermolysis bullosa acquisita Distinguish diseases from each other and from other immunobullous disease (eg, pemphigus and dermatitis herpetiformis) |
| Limitations: Clinical correlation necessary because the incidence of false positives, although rare, increases with age Note: This test does NOT include IgA BMZ antibody determination; linear IgA disease may be missed IgG BP180 and BP230 antibody levels are NOT included in this test, which may increase sensitivity in diagnosing pemphigoid |
| Follow-up: Perilesional skin biopsy by DIF is important in initial diagnosis (>90% of pemphigoid and epidermolysis bullosa acquisita cases are positive) |
| Test name: Bullous Pemphigoid (180 kDa & 230 kDa) Antigens, IgG |
| ARUP #: 0092566 |
| Methodology: Enzyme-Linked Immunosorbent Assay |
| Use: Test for IgG BP180 (BP Ag2) and BP230 (BP Ag1) antibodies in patients suspected or known to have any type of pemphigoid, including bullous pemphigoid and variant forms, mucous membrane (cicatricial) pemphigoid and herpes gestationis Detect relative levels of BP180 and BP230 IgG antibodies to diagnose pemphigoid and monitor disease activity including response to therapy |
| Limitations: 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 IgG BP180 and BP230 ELISAs are components of pemphigoid panel 0092001, which may be most useful for initial diagnosis of pemphigoid and monitoring response to therapy Correlations with IFA and DIF results and clinical presentation are important for initial diagnosis |
| Follow-up: Perilesional skin biopsy for DIF is important for diagnosis because of increased sensitivity (>90% of pemphigoid cases are positive); however, specificity is lower because of similar DIF findings in both pemphigoid and epidermolysis bullosa acquisita |