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ARUP Immunobullous Disease Testing Comparison

Immunobullous diseases are caused by or associated with specific autoantibodies that bind to epithelium in skin and mucous membranes. These disorders do not always show blistering and may present with common symptoms or signs, including itching, eczema, urticaria, or oral mucosal irritation. They may be better known as epithelial antibody-associated diseases. ARUP Laboratories offers a comprehensive selection of immunodermatology test panels to detect the autoantibodies associated with these diseases and, thus, aid in the diagnosis and monitoring of disease activity.

For more information about the immunodermatology panels offered by ARUP, refer to the Panel Tests table.

For more information about immunodermatology tests that aid in the diagnosis of specific epithelial antibody-associated diseases, refer to the Disease-Specific Tests table.

For more information about the individual tests that are included in the immunodermatology panels, refer to the Individual Tests table.

Test Guide Summary
ARUP Test Number and Mnemonic	ARUP Test Name	ARUP Panel Test Components
Serum Panels
3001409 IMBULDZPAN	Immunobullous Disease Antibody Panel (Preferred initial diagnostic panel when a patient presents with nonspecific symptoms and signs including pruritus, urticaria, eczema, and/or mucositis; with potentially overlapping epithelial antibody expression; or without distinguishing clinical features)	Basement Membrane Zone Antibody Panel (3001410) Pemphigus Antibody Panel, IgG (0090650) Pemphigus Antibodies, IgA by IIF (0092106)
3001410 BMZ AB PAN	Basement Membrane Zone Antibody Panel (Preferred initial diagnostic panel for patients with known BMZ antibodies detected, for example, by DIF testing on a biopsy specimen)	Pemphigoid Antibody Panel (0092001) Collagen Type VII Antibody, IgG by ELISA (2010905)
0092001 PGOID PAN	Pemphigoid Antibody Panel	Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF (0092056) Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF (0092057) Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA (0092566)
0090650 PEMPHI PAN	Pemphigus Antibody Panel, IgG (Preferred initial diagnostic panel for patients with known IgG cell surface antibodies detected, for example, by DIF testing on a biopsy specimen)	Cell Surface (Epithelial) Antibodies, IgG by IIF (0090266) Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA (0090649)
0090299 EPITHELIAL	Basement Membrane Zone and Cell Surface (Epithelial) Antibodies, IgG and IgA by IIF	Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF (0092056) Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF (0092057) Cell Surface (Epithelial) Antibodies, IgG by IIF (0090266) Pemphigus Antibodies, IgA by IIF (0092106)
3016534 PNP PLUS	Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA (Preferred expanded panel to aid in diagnosis and monitoring of paraneoplastic pemphigus)	Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF (0092107) Envoplakin Antibody, IgG by ELISA (3016533)
Specific Disease-Associated Serum Tests
0092106 IGA PEMPHI	Pemphigus Antibodies, IgA by IIF	No combination of tests
0092283 HG FACTOR	Pemphigoid Gestationis, Complement-Fixing Basement Membrane Antibodies (Herpes Gestationis Factor)	No combination of tests
2010902 EPI TRANS	Epidermal Transglutaminase (eTG/TG3) Antibody, IgA by ELISA	No combination of tests
0092107 PARA PEMPH	Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF	No combination of tests
Serum Test Panel Components and Individual Tests
0092056 EBMZ IGG	Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF	No combination of tests
0092057 EBMZ IGA	Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF	No combination of tests
0092566 BP180 230G	Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA	No combination of tests
2010905 COLLAG 7	Collagen Type VII Antibody, IgG by ELISA	No combination of tests
0090266 IGG EPI AB	Cell Surface (Epithelial) Antibodies, IgG by IIF	No combination of tests
0092106 IGA PEMPHI	Pemphigus Antibodies, IgA by IIF	No combination of tests
0090649 IGG DESMOG	Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA	No combination of tests
3016533 ENVO IGG	Envoplakin Antibody, IgG by ELISA	No combination of tests
Tissue Tests (Skin or Mucosa for DIF; Any Tissue or Organ for eMBP1)
0092572 CUTDIF 2012552 CUTDIF ADD	Direct Immunofluorescence, Tissue Biopsy (Cutaneous, Mucosal, Epithelial)	No combination of tests
2010921 EGMBP TISS	Eosinophil Granule Major Basic Protein, Tissue Biopsy	No combination of tests
For more information on assessing for immunobullous disease, refer to the ARUP Consult Immunobullous Diseases, Epithelial Antibody Associated topic.

Detailed Test Information

Panel Tests
Test Name and Number	Components Included (Separately Orderable)	Diseases With Associated Diagnostic Markers in the Test	Additional Notes
Immunobullous Disease Antibody Panel 3001409	Basement Membrane Zone Antibody Panel 3001410 Pemphigoid Antibody Panel 0092001 Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF 0092056 Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF 0092057 Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA 0092566 Collagen Type VII Antibody, IgG by ELISA 2010905 Pemphigus Antibody Panel, IgG 0090650 Cell Surface (Epithelial) Antibodies, IgG by IIF 0090266 Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA 0090649 Pemphigus Antibodies, IgA by IIF 0092106	Pemphigoid and pemphigoid variants, including nonbullous pemphigoid EBA and other IgG dermal (floor) localizing BMZ antibody-associated diseases Linear IgA disease, including linear IgA bullous dermatosis and chronic bullous disease of childhood Linear IgG/IgA bullous dermatosis Bullous lupus erythematosus Pemphigus foliaceus, pemphigus vulgaris, and other IgG-pemphigus variants Subcorneal pustular dermatosis, intraepidermal neutrophilic IgA dermatosis, and other IgA-pemphigus variants Intercellular IgG/IgA dermatosis	Serum IgG and IgA epithelial BMZ antibodies are assessed by IIF using human split-skin substrate (also known as salt-split skin), human intact skin substrate, and monkey esophagus substrate; when positive, limiting-dilution, end-point titers are reported ELISAs are performed with patient serum to determine IgG BP180, IgG BP230, IgG type VII collagen, IgG desmoglein 1, and IgG desmoglein 3 antibody levels Diagnostic tests are not currently available for antibodies to p200 (laminin gamma-1) or laminin-332, which show dermal (floor) localizing BMZ antibodies; anti-p200 pemphigoid may be the most common dermal (floor) localizing BMZ antibody-associated disease
Basement Membrane Zone Antibody Panel 3001410	Pemphigoid Antibody Panel 0092001 Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF 0092056 Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF 0092057 Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA 0092566 Collagen Type VII Antibody, IgG by ELISA 2010905	Pemphigoid and pemphigoid variants, including nonbullous pemphigoid EBA and other IgG dermal (floor) localizing BMZ antibody-associated diseases Linear IgA disease, including linear IgA bullous dermatosis and chronic bullous disease of childhood Linear IgG/IgA bullous dermatosis Bullous lupus erythematosus	Serum IgG and IgA epithelial BMZ antibodies are assessed by IIF using human split-skin substrate (also known as salt-split skin), human intact skin substrate, and monkey esophagus substrate; when positive, limiting-dilution, end-point titers are reported ELISAs are performed with patient serum to determine IgG BP180, IgG BP230, and IgG type VII collagen antibody levels Diagnostic tests are not currently available for antibodies to p200 (laminin gamma-1) or laminin-332, which show dermal (floor) localizing BMZ antibodies; anti-p200 pemphigoid may be the most common dermal (floor) localizing BMZ antibody-associated disease
Pemphigoid Antibody Panel 0092001	Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF 0092056 Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF 0092057 Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA 0092566	Pemphigoid and pemphigoid variants, including mucous membrane pemphigoid and nonbullous pemphigoid EBA and other IgG dermal (floor) localizing BMZ antibody-associated diseases Linear IgA disease, including linear IgA bullous dermatosis and chronic bullous disease of childhood Linear IgG/IgA bullous dermatosis	Serum IgG and IgA epithelial BMZ antibodies are assessed by IIF using human split-skin substrate (also known as salt-split skin), human intact skin substrate, and monkey esophagus substrate; when positive, limiting-dilution, end-point titers are reported ELISAs are performed with patient serum to determine IgG BP180 and IgG BP230 antibody levels
Pemphigus Antibody Panel, IgG 0090650	Cell Surface (Epithelial) Antibodies, IgG by IIF 0090266 Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA 0090649	Pemphigus foliaceus, pemphigus vulgaris, and other IgG-pemphigus variants	Serum IgG epithelial cell surface antibodies are assessed by IIF using human intact skin substrate and monkey esophagus substrate; when positive, limiting-dilution, end-point titers are reported ELISAs are performed with patient serum to determine IgG desmoglein 1, and IgG desmoglein 3 antibody levels IgA cell surface antibodies, characteristically positive in the rare IgA pemphigus variant, may be observed with IgG cell surface antibodies in nonclassical pemphigus variants
Basement Membrane Zone and Cell Surface (Epithelial) Antibodies, IgG and IgA by IIF 0090299	Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF 0092056 Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF 0092057 Cell Surface (Epithelial) Antibodies, IgG by IIF 0090266 Pemphigus Antibodies, IgA by IIF 0092106	Pemphigoid and pemphigoid variants, including nonbullous pemphigoid EBA and other IgG dermal (floor) localizing BMZ antibody-associated diseases Linear IgA disease, including linear IgA bullous dermatosis and chronic bullous disease of childhood Linear IgG/IgA bullous dermatosis Bullous lupus erythematosus Pemphigus foliaceus, pemphigus vulgaris, and other IgG-pemphigus variants Subcorneal pustular dermatosis, intraepidermal neutrophilic IgA dermatosis, and other IgA-pemphigus variants Intercellular IgG/IgA dermatosis	Serum IgG and IgA epithelial BMZ and cell surface antibodies are assessed by IIF using human split-skin substrate (also known as salt-split skin), human intact skin substrate, and monkey esophagus substrate; when positive, limiting-dilution, end-point titers are reported Enhanced diagnostic sensitivity is achieved with concomitant ELISA testing, also useful for following disease activity Positive IIF limiting-dilution, end-point titers may be useful to follow disease activity, both with ELISA antibody levels and when relevant ELISAs are unavailable or negative
Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA 3016534	Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF 0092107 Envoplakin Antibody, IgG by ELISA 3016533	Paraneoplastic pemphigus Paraneoplastic autoimmune multiorgan syndrome	Serum IgG BMZ and epithelial cell surface/intercellular substance antibodies are assessed by IIF using rodent substrates (including rat bladder and mouse bladder) and monkey esophagus substrate; when positive on rodent substrates, limiting-dilution, end-point titers are reported Results are supported by IIF for IgG antibodies that is performed on intercalated discs using rodent heart and portal tracts using rodent liver Positive IIF test results indicate IgG serum antibodies to multiple epithelia (simple, columnar, transitional) with several possible epithelial targets: predominantly plakins (eg, envoplakin, periplakin, desmoplakin I, desmoplakin II, epiplakin, plectin, BP230) but also cadherins (eg, desmoglein 1, desmoglein 3, desmocollin 1, desmocollin 2, desmocollin 3), A2ML1, laminin-332, and/or BP180; therefore, other targeted testing for specific epithelial antibodies can be helpful IgG envoplakin antibody levels correlate with the extent of mucocutaneous disease in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome), but additional or different epithelial antigens may be antibody targets ELISA testing with IIF may increase diagnostic sensitivity

Disease-Specific Tests
Test Name and Number	Test Inclusions (Not Separately Orderable)	Diseases With Associated Diagnostic Markers in the Test	Additional Notes
Pemphigus Antibodies, IgA by IIF 0092106	n/a	IgA pemphigus Nonclassical pemphigus subtypes Intercellular IgG/IgA dermatosis	Serum IgA epithelial cell surface antibodies are assessed by IIF using monkey esophagus substrate and human intact skin with limiting-dilution, end-point titers when positive IgA pemphigus is a rare disease with certain clinical and histopathologic variants
Pemphigoid Gestationis, Complement-Fixing Basement Membrane Antibodies (Herpes Gestationis Factor) 0092283	Complement-Fixing Basement Membrane Zone Antibodies Bullous Pemphigoid (BP180) Antibody, IgG by ELISA Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF	Pemphigoid gestationis Other immunobullous diseases with complement-fixing BMZ antibodies	Serum complement-fixing BMZ antibodies and IgG BMZ antibodies are assessed by IIF using human split-skin substrate (also known as salt-split skin) Pemphigoid gestationis is a rare disease of pregnancy and the puerperium; additional testing should also be considered
Epidermal Transglutaminase (eTG/TG3) Antibody, IgA by ELISA 2010902	n/a	Dermatitis herpetiformis with and without celiac disease	IgA eTG, also known as TG3 antibodies, are pathogenic in dermatitis herpetiformis An increased level of IgA TG3 antibodies is distinctly characteristic of dermatitis herpetiformis and levels correlate with disease severity; however, a normal level does not rule out dermatitis herpetiformis Dermatitis herpetiformis is associated with celiac disease in most patients; celiac disease serologies are also recommended
Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Screening Antibodies by IIF 0092107	IgG cell surface antibodies by IIF IgG BMZ antibodies by IIF	Paraneoplastic pemphigus Paraneoplastic autoimmune multiorgan syndrome	Serum IgG BMZ and epithelial cell surface antibodies are assessed by IIF using rodent substrates (including rat bladder and mouse bladder) and monkey esophagus substrate; when positive on rodent substrates, limiting-dilution, end-point titers are reported Results are supported by IIF for IgG antibodies that is performed on intercalated discs using rodent heart and portal tracts using rodent liver; IgA paraneoplastic pemphigus antibody IIF using rodent substrates can be performed upon request Positive test results indicate IgG serum antibodies to multiple epithelia (simple, columnar, transitional) with several possible epithelial targets: predominantly plakins (eg, envoplakin, periplakin, desmoplakin I, desmoplakin II, epiplakin, plectin, BP230), but also cadherins (eg, desmoglein 1, desmoglein 3, desmocollin 1, desmocollin 2, desmocollin 3), A2ML1, laminin-332, and/or BP180 Envoplakin Antibody, IgG by ELISA and other targeted testing for specific epithelial antibodies can be helpful
A2ML1, alpha (α)-2-macroglobulin-like-1; BMZ, basement membrane zone; EBA, epidermolysis bullosa acquisita; ELISA, enzyme-linked immunosorbent assay; eMBP1, eosinophil granule major basic protein 1; eTG, epidermal transglutaminase; IgA, immunoglobulin A; IgG, immunoglobulin G; IIF, indirect immunofluorescence; TG3, transglutaminase 3

Individual Tests
Test Name and Number	Panels Containing Test as Component	Diseases with Associated Diagnostic Markers in the Test	Additional Notes
Basement Membrane Zone (Epithelial) Antibodies, IgG by IIF 0092056	Immunobullous Disease Antibody Panel 3001409 Basement Membrane Zone Antibody Panel 3001410 Pemphigoid Antibody Panel 0092001	Pemphigoid and pemphigoid variants/subtypes EBA and other IgG dermal (floor) localizing BMZ antibody-associated diseases	Serum IgG BMZ antibodies are assessed by IIF using monkey esophagus substrate and human split-skin substrate (also known as salt-split skin) and, when positive, include epidermal (roof), dermal (floor), or combined epidermal-dermal (roof and floor) localization patterns IgG BMZ antibodies may be coexpressed with IgA BMZ antibodies
Basement Membrane Zone (Epithelial) Antibodies, IgA by IIF 0092057	Immunobullous Disease Antibody Panel 3001409 Basement Membrane Zone Antibody Panel 3001410 Pemphigoid Antibody Panel 0092001	Linear IgA disease, including linear IgA bullous dermatosis, chronic bullous disease of childhood, and immunobullous disease of childhood IgA variant EBA	Serum IgA BMZ antibodies are assessed by IIF using monkey esophagus substrate and human split-skin substrate (also known as salt-split skin) and, when positive, include epidermal (roof), dermal (floor), or combined epidermal-dermal (roof and floor) localization patterns IgA BMZ antibodies may be coexpressed with IgG BMZ antibodies
Bullous Pemphigoid (BP180 and BP230) Antibodies, IgG by ELISA 0092566	Immunobullous Disease Antibody Panel 3001409 Basement Membrane Zone Antibody Panel 3001410 Pemphigoid Antibody Panel 0092001	Pemphigoid and pemphigoid variants, including nonbullous pemphigoid	IgG BP180 antibody levels correlate with disease activity in some patients with pemphigoid IgG BP180 antibody levels characteristically are increased in pemphigoid gestationis and may remain increased even with disease remission
Collagen Type VII Antibody, IgG by ELISA 2010905	Immunobullous Disease Antibody Panel 3001409 Basement Membrane Zone Antibody Panel 3001410	EBA Bullous lupus erythematosus	IgG type VII collagen antibody levels correlate with disease activity in some patients with EBA
Cell Surface (Epithelial) Antibodies, IgG by IIF 0090266	Immunobullous Disease Antibody Panel 3001409 Pemphigus Antibody Panel, IgG 0090650	IgG pemphigus variants, including pemphigus foliaceus and pemphigus vulgaris	Serum IgG cell surface antibodies are assessed by IIF using intact human skin substrate and monkey esophagus substrate, with and without increased IgG desmoglein 1 and/or IgG desmoglein 3 antibody levels Levels of IgG cell surface antibodies correlate with disease activity
Desmoglein 1 and Desmoglein 3 (Pemphigus) Antibodies, IgG by ELISA 0090649	Immunobullous Disease Antibody Panel 3001409 Pemphigus Antibody Panel, IgG 0090650	Pemphigus foliaceus Fogo selvagem (endemic pemphigus foliaceus) Pemphigus erythematosus Pemphigus vulgaris Pemphigus vegetans Pemphigus herpetiformis	An increased IgG desmoglein 1 antibody level with normal IgG desmoglein 3 antibody level correlates with disease activity in pemphigus foliaceus A predominantly increased IgG desmoglein 3 antibody level with or without an increased IgG desmoglein 1 antibody correlates with disease activity in pemphigus vulgaris IgG antibody expression to both desmogleins 1 and 3 is associated with skin and mucosal lesions characteristic of pemphigus foliaceus and pemphigus vulgaris and correlates with disease activity
Envoplakin Antibody, IgG by ELISA 3016533	Paraneoplastic Pemphigus (Paraneoplastic Autoimmune Multiorgan Syndrome) Expanded Antibody Panel by IIF With ELISA 3016534	Paraneoplastic pemphigus Paraneoplastic autoimmune multiorgan syndrome	IgG envoplakin antibody levels correlate with the extent of mucocutaneous disease in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome), but additional or different epithelial antigens may be antibody targets IgG envoplakin antibody levels can be increased in other IgG pemphigus variants ELISA testing may be more sensitive than IIF
A2ML1, alpha (α)-2-macroglobulin-like-1; BMZ, basement membrane zone; DIF, direct immunofluorescence; EBA, epidermolysis bullosa acquisita; ELISA, enzyme-linked immunosorbent assay; eMBP1, eosinophil granule major basic protein 1; eTG, epidermal transglutaminase; IgA, immunoglobulin A; IgG, immunoglobulin G; IIF, indirect immunofluorescence; n/a, not applicable; TG3, transglutaminase 3

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