Immunobullous Diseases, Epithelial Antibody Associated

Immunobullous diseases are caused by or associated with specific autoantibodies that bind to epithelium.  The autoantibodies are directed to components in skin and mucous membranes that are critical for cell-cell and cell-matrix adhesion, resulting directly or indirectly in tissue separation/blistering within and beneath the epithelium.  Immunobullous diseases include pemphigus, pemphigoid, pemphigoid gestationis, epidermolysis bullosa acquisita (EBA), linear IgA disease, dermatitis herpetiformis, and bullous lupus erythematosus. Although the various diseases have characteristic clinical and histopathologic features, presentation may be atypical and often demonstrates overlap with other immunobullous diseases or with more common skin diseases such as eczema and urticaria. Accurate diagnosis depends on clinical assessment plus the detection of autoantibodies (tissue bound and/or circulating).     Direct immunofluorescence (DIF) microscopy of a perilesional biopsy is used to detect patterns of immunoglobulins and complement component 3 (C3) that characterize the diseases. DIF is sensitive and is considered a diagnostic standard.   Serum antibody testing using indirect fluorescent antibody (IFA) testing and enzyme-linked immunosorbent assays (ELISAs) can be used to distinguish the various disorders more precisely based on autoantibody reactions to specific epithelial antigenic targets.   Although DIF testing of a biopsy specimen may be more sensitive than serum testing for diagnosing immunobullous diseases, either may be positive when the other is negative, and together they offer the most sensitivity. Precise diagnosis is key for prognosis, treatment decisions, and, importantly, because some immunobullous skin diseases are associated with malignancies.  Monitoring serum autoantibody profiles and levels may be useful to follow disease expression and activity, including response to therapy.

Quick Answers for Clinicians

How are the various immunobullous diseases that affect skin and mucous membranes differentiated?

Specific autoantibodies develop in patients with autoimmune blistering diseases. These antibodies show different staining patterns by direct immunofluorescence (DIF) in perilesional tissue biopsy specimens based on reactivity with certain adhesion components that are detected in serum antibodies. For example, desmoglein 1 and 3 autoantibodies in serum are associated with pemphigus, which shows epithelial cell surface staining by DIF in a biopsy specimen, whereas BP 180 and BP 230 autoantibodies are associated with pemphigoid, which shows epithelial basement membrane zone staining by DIF in a biopsy specimen. See Primary ​Autoantigens Associated with Specific Immunobullous Skin Diseases table for more information.

Which tests are useful for monitoring immunobullous diseases?

Serum antibody profiles and levels often correlate with disease expression and activity in immunobullous diseases; therefore, serologic testing using indirect fluorescent antibody (IFA) tests and enzyme-linked immunosorbent assays (ELISAs) enables monitoring of disease and treatment response.  

Where can I find more information on testing for specific immunobullous diseases?

ARUP Consult has additional information on the following diseases: pemphigus, pemphigoid, pemphigoid gestationis, epidermolysis bullosa acquisita (EBA), linear IgA disease, dermatitis herpetiformis, and paraneoplastic pemphigus. It also has algorithms that show testing steps for the various diseases.

Indications for Testing

Laboratory testing for immunobullous diseases is appropriate in the following contexts:

  • Diagnosis of immunobullous diseases (epithelial antibody-associated diseases) in patients with blisters, bullae, or vesicles and erosive lesions of skin and/or mucous membranes,    often accompanied by itching and secondary lesions; also may present as eczema, urticaria, or pruritus without blistering
  • Monitoring of disease activity after established diagnosis of an immunobullous disease

Disease-specific indications and clinical features are listed in the table below.

Disease-Specific Indications and Features
Pemphigus

Pemphigus vulgarisa (mucosal dominant, mucocutaneous, or cutaneous)

Fragile, flaccid bullae that evolve into erosions, crusting

Involvement of mucosa or skin, or both

Nikolsky sign

Pemphigus vegetans (variant of pemphigus vulgaris)

Erythematous, vegetating intertriginous plaques

Pemphigus foliaceusa

Superficial bullae, erosions, and hyperkeratotic scales with crusting

Nikolsky sign

Sparing of mucus membranes

Often in distribution of seborrheic dermatitis

Pemphigus erythematosus (variant of pemphigus foliaceus with features of lupus erythematosus)

Superficial erosions, erythema, crusting, often of malar and seborrheic areas

Also known as Senear-Usher syndrome

Endemic pemphigus (variant of pemphigus foliaceus with genetic and environmental cofactors, primarily in Brazil; also known as fogo selvagem)

Superficial erosions, erythema, crusting, localized to seborrheic areas, head, and upper chest

Generalized, erythrodermic presentation also observed

Pemphigus herpetiformis

Erythematous, bullous, vesicular, pustular, or papular lesions, typically with severe pruritus and in a herpetiform pattern

Mucosal involvement is uncommon

IgA pemphigus

Fragile blisters filled with fluid that evolve into pustules; pruritus

Mucosal involvement is uncommon

Two major subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis

Paraneoplastic pemphigus

Various possible lesion types, including flaccid and/or tense bullae, erosions, urticarial lesions, erythema multiformelike lesions, lichen planus-like lesions, flat scaly papules

Mucosal involvement, often oral and ocular

Pulmonary and gastrointestinal involvement

Pemphigoid

Bullous pemphigoid and variants (urticarial, localized, drug induced)b

Tense bullae and erosions, prominent pruritus (variant forms may not have bullae)

Possible erythematous or urticarial lesions, eczematous dermatitis, pruritus without blisters

Association with neurologic disease (eg, Parkinson disease and multiple sclerosis)

Mucous membrane pemphigoid (cicatricial, ocular, Brunsting-Perry, and antilaminin-332, formerly known as antiepiligrin)

Recurrent bullae affecting mucous membranes as well as skin, with erosions, scarring sequelae

Primarily ocular and oral involvement; Brunsting-Perry variant demonstrates head and neck blistering without mucosal lesions; up to 30% of antilaminin-332 pemphigoid cases have associated malignancy

Anti-p200 pemphigoid (anti-laminin γ1)

Bullae similar to those seen in bullous pemphigoid, vesicles, and urticarial plaques; also palmoplantar, cephalic, and mucosal involvement; frequent development of scars/milia; associated with psoriasis in approximately 30% of cases

Pemphigoid gestationis (previously called herpes gestationis)

Pruritic urticarial papules and bullae on abdomen and trunk that occur during pregnancy or postpartum period; may flare with menses and/or hormonal treatment

EBA

Variants (classical/mechanobullous; nonclassical/nonmechanobullous with features overlapping with bullous pemphigoid; mucous membrane EBA predominantly affects mucosa with squamous epithelia; Brunsting-Perry type involves lesions primarily of head and neck; IgA EBA presents with linear IgA deposits, instead of IgG, and clinical similarity to linear IgA disease, but may have more severe scarring sequelae)

Skin fragility, tense bullae, vesicles, and erosions (in classic or mechanobullous type); healing with scarring and milia

Tense bullae on erythematous, urticarial base (in inflammatory type)

Linear IgA diseasec (Linear IgA bullous dermatosis in adults; chronic bullous disease of childhood in children)

Subepidermal bullae involving skin and adjacent mucus membranes

Tense blisters on skin with “necklacelike” or “string-of-pearls” arrangement

Oral mucosal involvement (common in adult disease) with ulcers and/or erosions

Possible annular or multiformelike lesions

Dermatitis herpetiformis

Grouped vesicles, papules, mainly on elbows, knees, buttocks; primary lesions may be replaced by secondary lesions due to profound pruritus and consequent scratching

Often presents with symmetrical cutaneous involvement

Association with intestinal gluten sensitivity/celiac disease

Bullous systemic lupus erythematosus

Nonscarring blisters on erythematous or normal skin

Lesions on skin exposed to sun

Lichen planus pemphigoides Lichen planus-like papules with blisters, skin and/or mucosal involvement

aPemphigus vulgaris and pemphigus foliaceus can also be drug induced; implicated drugs include thiol-containing medications, masked thiols, enalapril, and dipyrone.

bPemphigoid is idiopathic in most cases but a minority of cases are drug induced; implicated drugs include antibiotics, ACE inhibitors, sulfasalazine, phenacetin, DPP4is, and checkpoint inhibitors (PD-1/PD-L1 monoclonal antibodies used in treatment for lung cancer and melanoma).

cMost cases of linear IgA disease are idiopathic but some are drug induced; implicated drugs include antibiotics, especially vancomycin, cardiac drugs, NSAIDs, chemotherapeutic agents, antiseizure medications, glibenclamide, diethylcarbamazine, cyclosporine, and lithium.

ACE, angiotensin-converting enzyme; DPP4is, dipeptidyl peptidase-4 inhibitors; γ, gamma; NSAIDs, nonsteroidal anti-inflammatory drugs

Sources: Witte, 2018 ; Otten, 2014 ; Baum, 2014 ; Patricio, 2009 ; Stinco, 2005 ; Kridin, 2019 

Laboratory Testing​

Autoantibody Testing

Serology

In serologic testing for immunobullous skin diseases, the patient serum is incubated with epithelial tissue substrates to determine if there are antibodies in the circulation that target particular surfaces in the substrates being tested. IFA tests and ELISAs are both useful serologic tests for these diseases.  IFA testing enables detection of circulating antibodies and deposition patterns, whereas ELISAs both identify and semiquantify antibodies.  ELISAs have been developed to detect antibodies to antigens common in (but not exclusive to) pemphigus vulgaris and pemphigus foliaceus, bullous pemphigoid and pemphigoid variants, EBA, and other diseases. In addition to ELISAs, immunoblotting and immunoprecipitation are sensitive and specific testing methods that provide serologic confirmation of immunobullous skin diseases, but these methods are mainly research procedures and are not readily available for diagnostic testing.  

Serum antibody profiles and titers detected by IFA and serum antibody levels as determined by ELISAs correlate with immunobullous disease manifestations and can be used to monitor disease expression and activity. This correlation is particularly relevant in IgG pemphigus variants, including pemphigus foliaceus and pemphigus vulgaris.

Immunohistopathology​

DIF microscopy of a perilesional biopsy is useful to detect tissue-bound autoantibody or C3 deposition.    (Recent research provides clarification about the diagnostic sensitivity of specific perilesional biopsy locations. ) This testing is considered a diagnostic standard for immunobullous diseases.  The nature and pattern of autoantibody deposition assists in distinguishing the diseases.  For example, in IgG variant pemphigus, IgG and C3 are deposited in a netlike or “honeycomb” pattern in the epidermis/epithelium, and, in pemphigoid, IgG and C3 are deposited in a bandlike, linear pattern along the basement membrane zone. 

Autoantigens Common to Specific Diseases

The table below provides information about the main autoantigens associated with specific immunobullous skin diseases.

Primary Autoantigens Associated with Specific Immunobullous Skin Diseases
Pemphigus
Pemphigus vulgaris Desmoglein 3 with or without desmoglein 1
Pemphigus foliaceus Desmoglein 1
Pemphigus erythematosus Desmoglein 1 and/or desmoglein 3 (antinuclear antibodies also are detected)
Pemphigus vegetans Desmoglein 3 with or without desmoglein 1, desmocollin 3
IgA pemphigus Desmocollin 1, desmocollin 2, desmocollin 3, desmoglein 1 and/or desmoglein 3
Paraneoplastic pemphigus Desmoglein 1, desmoglein 3, desmoplakin 1, desmoplakin 2, envoplakin, periplakin, epiplakin, BP 230, alpha-2-macroglobulin-like-protein 1, plectin, and desmocollin 1, desmocollin 2, and desmocollin 3
Pemphigoid
Bullous pemphigoid BP 180, BP 230
Mucus membrane pemphigoid BP 180, p200 (laminin γ1), laminin-332, α6β4 integrin
Anti-p200 pemphigoid p200 (laminin γ1)
Pemphigoid gestationis (formerly called herpes gestationis) BP 180 (rarely also BP 230)
EBA Type VII collagen
Linear IgA disease LAD-1 (BP 180), BP 230
Dermatitis herpetiformis Tissue transglutaminase (also known as TG2) and epidermal transglutaminase (also known as TG3)

α, alpha; β, beta; TG2, transglutaminase type 2; TG3, transglutaminase type 3

Sources: Otten, 2014 ; Kridin, 20019 

Additional Disease-Specific Testing Information

See individual ARUP Consult topics for specific testing recommendations for various immunobullous diseases.

Monitoring

Serum antibody testing using IFA and ELISA enables monitoring of disease activity. Patterns and relative antibody titers by IFA are useful to determine expression profiles that relate to disease manifestations. Autoantibody levels as determined by ELISAs often correlate with disease activity and are useful in monitoring response to therapy after an established diagnosis.  

Tests for Monitoring Treatment Response in Specific Immunobullous Diseases
Disease Tests Comments
Pemphigoid Pemphigoid Antibody Panel - Epithelial Basement Membrane Zone Antibodies, IgG and IgA, BP 180 and BP 230 Antibodies, IgG Testing for BP 180 and BP 230 antibodies (IgG) by ELISA may be appropriate in some patients to monitor disease activity; substitute testing with pemphigoid antibody panel (includes IgG BP 180 and IgG BP 230 antibody levels) intermittently to detect changing antibody patterns, which may have implications for disease expression
Pemphigus (foliaceus, erythematosus, vulgaris, or vegetans) Pemphigus Antibody Panel - Epithelial Cell Surface Antibodies and Desmoglein 1 and Desmoglein 3 Antibodies, IgG Testing for desmoglein 1 and desmoglein 3 antibodies (IgG) by ELISA may be appropriate in some patients to monitor disease activity; substitute testing with pemphigus antibody panel (includes IgG desmoglein 1 and IgG desmoglein 3 antibody levels) intermittently to detect changing antibody patterns, which may have implications for disease expression
IgA pemphigus Pemphigus Antibody IgA Testing for IgA cell surface antibodies may be appropriate, not only for IgA pemphigus, but to monitor disease activity in nonclassical pemphigus presentations with both IgA and IgG cell surface antibodies
Paraneoplastic pemphigus Paraneoplastic Pemphigus Antibody Screen and, if increased, Desmoglein 1 and Desmoglein 3 Antibodies, IgG ELISA and/or BP 180 and BP 230 Antibodies, IgG ELISA
EBA Epithelial Basement Membrane Zone Antibody IgG AND Collagen Type VII Antibody IgG by ELISA

OR

Epithelial Skin Antibody AND Collagen Type VII Antibody IgG by ELISA

OR

Basement Membrane Zone Antibody Panel

Testing for type VII collagen antibody (IgG) by ELISA may be appropriate in some patients to monitor disease activity; intermittently test with epithelial skin antibody panel and type VII collagen antibody (IgG) by ELISA or basement membrane zone antibody panel to detect changing antibody patterns as well as levels, which may have implications for disease expression
Linear IgA disease Epithelial Basement Membrane Zone Antibody IgA

OR

Epithelial Skin Antibody

Dermatitis herpetiformis Celiac Disease Reflexive Cascade and Epidermal Transglutaminase (etG/tTG3) Antibody, IgA by ELISA
Pemphigoid gestationis Herpes Gestationis Factor (Complement-Fixing Basement Membrane Zone Antibody IgG)

ARUP Laboratory Tests

Additional detail about each test listed below can be found in the ARUP Laboratory Test Directory (LTD). In addition, each test name links directly to the LTD.

Immunohistopathologic Test (DIF)

Perform along with serum antibody testing and formalin-fixed tissue histopathology for assessment of pruritic, urticarial, blistering, crusted, and/or erosive disorders (immunobullous diseases with or without blistering); perilesional specimen recommended for blisters and/or erosions

Correlate with histopathology examination of formalin-fixed tissue to assess inflammation in immune-mediated cutaneous diseases (such as lupus erythematosus, vasculitis, lichen planus); lesional specimen recommended

Optimal specimen location varies according to disease type, blistering or nonblistering

Components: cutaneous DIF, biopsy; DIF includes IgG, IgG4, IgM, IgA, C3, and fibrinogen with diagnostic interpretation of staining patterns

Recommended Serologic Tests
Semiquantitative IFA and Semiquantitative ELISA (With Biopsy DIF in Initial Assessment)

Use as initial testing panel to aid in diagnosis of and to distinguish among skin and mucous membrane disorders that present with blistering, erosions, crusts, eczema, pruritus, and/or urticaria, including pemphigoid and pemphigus variants, EBA, and linear IgA disease

Semiquantitative IFA and ELISA; positive limiting-dilution end-point antibody titer(s) (IFA) and antibody level in units (ELISA)

Components: IFA for IgG and IgA basement membrane zone antibodies (pemphigoid, EBA, linear IgA disease), includes basement membrane zone split-skin substrate (also known as salt split skin), and IgG and IgA cell surface antibodies (IgG and IgA pemphigus subtypes, nonclassical pemphigus, intercellular IgG/IgA dermatosis); ELISAs for IgG antibodies to BP 180, BP 230, type VII collagen, desmoglein 1, and desmoglein 3

Use as the preferred initial diagnostic panel for skin and mucous membrane disorders that present with blistering, erosions, crusts, eczema, pruritus, and/or urticaria from suspected basement membrane zone antibody-associated disease, including pemphigoid, EBA, linear IgA disease, and bullous lupus erythematosus

Semiquantitative IFA and ELISA; positive limiting-dilution end-point antibody titer(s) (IFA) and antibody level in units (ELISA)

Alternatively, order Immunobullous Disease Panel, Epithelial, for initial diagnostic assessment

Components: IFA for IgG and IgA basement membrane zone antibodies (pemphigoid, EBA, linear IgA disease), includes basement membrane zone split-skin substrate (also known as salt split skin); ELISAs for IgG antibodies to BP 180, BP 230, and type VII collagen (all components included in immunobullous disease panel)

Use as preferred serum antibody panel for initial assessment and disease monitoring in IgG-variant pemphigus

Semiquantitative IFA and ELISA; positive limiting-dilution end-point antibody titer(s) (IFA) and antibody level in units (ELISA)

To aid in diagnosis of pemphigus along with other possible immunobullous diseases, order concurrently with the basement membrane zone antibody panel and the pemphigus antibody IgA test

The Immunobullous Disease Panel, Epithelial, can be ordered as an alternative to this test

Components: IFA for IgG epithelial cell surface antibodies; ELISAs for IgG antibodies to desmoglein 1 and IgG desmoglein 3 (all components included in immunobullous disease panel)

Use as general initial test for immunobullous diseases or to monitor EBA and linear IgA disease expression (test does not include ELISAs for disease-related antibodies to specific antigenic targets)

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

Consider ordering with ELISAs for IgG bullous pemphigoid antigens, BP 180 and BP 230, and IgG type VII collagen antibody for suspected pemphigoid or EBA, and/or IgG desmoglein 1 and IgG desmoglein 3 antibodies for suspected pemphigus; alternatively, consider the Immunobullous Disease Panel, Epithelial

For more sensitive and specific pemphigoid or pemphigus testing, refer to antibody panels for pemphigus, basement membrane zone, or pemphigoid

Components: IFA for IgG and IgA basement membrane zone antibodies (pemphigoid, EBA, linear IgA disease) and IgG and IgA cell surface antibodies (IgG and IgA pemphigus subtypes, nonclassical pemphigus, and intercellular IgG/IgA dermatosis) (all components included in immunobullous disease panel)

Use as antibody panel for initial assessment and disease monitoring in paraneoplastic pemphigus

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

Consider ordering concurrently with Immunobullous Disease Panel, Epithelial, or ELISAs for IgG desmoglein 1 and IgG desmoglein 3 antibodies and IgG bullous pemphigoid antigens, BP 180 and BP 230 antibodies; any of these epithelial antibodies may be increased in paraneoplastic pemphigus

If other, more common, types of pemphigus are of diagnostic consideration, order antibody panel test for pemphigus first or concurrently with this test

If IgA paraneoplastic antibody testing is indicated, contact ARUP Client Services to request testing

Components: IFA for IgG epithelial antibodies on rodent substrates; IgG cell surface and basement membrane antibodies on rat and mouse bladder substrates, intercalating disks on mouse heart substrate, portal tracts on mouse liver substrate

Use as antibody test for initial assessment and disease monitoring of dermatitis herpetiformis and to discriminate among the immunobullous skin diseases

Quantitative nephelometry, semiquantitative IFA and ELISA; IgA mg/dL, positive limiting-dilution end-point antibody titer(s) (IFA) and antibody level in units (ELISA)

Use with epidermal transglutaminase antibody, IgA, and with basement membrane zone and pemphigus panel tests or with Immunobullous Disease Panel, Epithelial, and epidermal transglutaminase antibody, IgA testing for dermatitis herpetiformis or other concurrent immunobullous disease

Components: quantitative nephelometry for immunoglobulin A with one or more reflexive tests that may include semiquantitative IFA for IgA endomysial antibodies; semiquantitative ELISAs for IgA and IgG antibodies to tissue transglutaminase and deamidated gliadin peptide (DGP)

Use to assess and monitor disease in patients with possible dermatitis herpetiformis

Semiquantitative ELISA; antibody level in units

For initial diagnosis and assessment of disease progression/changes and to distinguish from other immunobullous diseases with epithelial antibodies, order with Celiac Disease Reflexive Cascade and Immunobullous Disease Panel, Epithelial, OR with Celiac Disease Reflexive Cascade and Basement Membrane Zone Antibody Panel and/or pemphigus antibody panel, IgG

Component: ELISA for IgA antibodies to epidermal transglutaminase (eTG), also known as transglutaminase type 3 or TG3

Use as antibody panel for initial assessment and disease monitoring in pemphigoid and linear IgA bullous dermatosis and to discriminate among the immunobullous skin diseases with epithelial basement membrane zone antibodies

Semiquantitative IFA and ELISA; positive limiting-dilution end-point antibody titer(s) (IFA) and antibody level in units (ELISA)

Alternatively, order Basement Membrane Zone Antibody Panel or Immunobullous Disease Panel, Epithelial

Components: IFA for IgG and IgA basement membrane zone antibodies (pemphigoid, EBA, linear IgA disease), includes basement membrane zone split-skin substrate (also known as salt split skin); ELISAs for IgG antibodies to BP 180 and BP 230 (all components included in the immunobullous disease panel and in the basement membrane zone antibody panel, but this panel does not include IgG antibodies to type VII collagen ELISA that is included in others)

Other Serologic Tests
Semiquantitative IFA and Semiquantitative ELISA

Use to assess and monitor positive IgA basement membrane zone antibodies in patients with linear IgA disease, including linear IgA bullous dermatosis and chronic bullous disease of childhood, IgA variant EBA, and overlap expression in pemphigoid with IgG basement membrane zone antibodies

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

Consider ordering concurrently with IgG epithelial basement membrane zone antibody testing, bullous pemphigoid antigens, BP 180 and BP 230 antibodies, and/or IgG collagen type VII antibody; alternatively, order Basement Membrane Zone Antibody Panel or Immunobullous Disease Panel, Epithelial

Components: IFA for IgA basement membrane zone antibodies (included in the immunobullous disease panel, the basement membrane zone antibody panel, the pemphigoid antibody panel, and the epithelial skin antibody panel)

Use to assess and monitor positive IgG basement membrane zone antibodies in patients with pemphigoid and EBA, especially those with normal IgG BP 180, IgG BP 230, and IgG type VII collagen antibody levels

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

Consider ordering concurrently with IgA epithelial basement membrane zone antibody testing, bullous pemphigoid antigens, BP 180 and BP 230 antibodies, and/or IgG collagen type VII antibody; alternatively, order Basement Membrane Zone Antibody Panel or Immunobullous Disease Panel, Epithelial

Components: IFA for IgG basement membrane zone antibodies (included in the immunobullous disease panel, the basement membrane zone antibody panel, the pemphigoid antibody panel, and the epithelial skin antibody panel)

Use to monitor disease in patients diagnosed with pemphigoid and increased IgG BP 180 and/or BP 230 antibody levels; IgG BP 180 antibody levels correlate with disease activity in some patients

Semiquantitative ELISA; antibody level in units

For initial diagnosis and assessment of disease progression/changes and intermittent monitoring, preferred test is Basement Membrane Zone Antibody Panel, or Immunobullous Disease Panel, Epithelial

Components: ELISAs for IgG antibodies to bullous pemphigoid antigens, BP 180 and BP 230 (included in the immunobullous disease panel, the basement membrane zone antibody panel, and the pemphigoid antibody panel)

Use to monitor disease in patients diagnosed with EBA or bullous lupus erythematosus and increased IgG type VII collagen antibody levels

Semiquantitative ELISA; antibody level in units

For initial diagnosis, preferred test is Immunobullous Disease Panel, Epithelial, or Basement Membrane Zone Antibody Panel

For assessment of disease progression/changes and intermittent monitoring, consider ordering concurrently with epithelial basement membrane zone antibody IgG and bullous pemphigoid antigens, BP 180 and BP 230 IgG antibodies

Components: ELISA for IgG antibodies to type VII collagen (included in the immunobullous disease panel and in the basement membrane zone antibody panel)

Use to monitor disease in patients diagnosed with pemphigus and increased IgG desmoglein 1 and/or 3 antibodies; antibody levels correlate with disease activity

Semiquantitative ELISA; antibody level in units

Decreased diagnostic sensitivity and specificity compared with pemphigus antibody panel; consider ordering with IgG epithelial cell surface antibody test

For initial diagnosis and assessment of disease progression/changes and intermittent monitoring, preferred test is pemphigus antibody panel testing or Immunobullous Disease Panel, Epithelial

Components: ELISAs for IgG antibodies to desmoglein 1 and desmoglein 3 (included in the immunobullous disease panel and in the pemphigus antibody panel, IgG)

Use to assess and monitor patients with positive IgG cell surface antibodies in pemphigus, especially in patients with normal IgG desmoglein 1 and IgG desmoglein 3 antibody levels

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

Decreased diagnostic sensitivity and specificity compared with pemphigus antibody panel; consider ordering with IgG desmoglein 1 and IgG desmoglein 3 antibodies

For initial diagnosis and disease monitoring, preferred test is pemphigus antibody panel or Immunobullous Disease Panel, Epithelial

Components: IFA for IgG cell surface antibodies (included in the immunobullous disease panel, the pemphigus antibody panel, IgG, and the epithelial skin antibody panel)

Use to assess and monitor patients with possible IgA pemphigus, a rare disease with certain clinical and histopathologic subtypes

Semiquantitative IFA; positive limiting-dilution end-point antibody titer(s)

If other types of pemphigus are of diagnostic consideration, including nonclassical pemphigus and intercellular IgG/IgA dermatosis, order this test concurrently with panel test for IgG pemphigus antibodies, or, for broader epithelial antibody testing, order Immunobullous Disease Panel, Epithelial

Components: IFA for IgA cell surface antibodies (included in the immunobullous disease panel and the epithelial skin antibody panel)

Medical Experts

Contributor

Leiferman

Picture of Kristin M. Leiferman, MD

 

Co-Director, Immunodermatology Laboratory, Professor of Dermatology, and Adjunct Professor of Pathology, University of Utah
Medical Director, Immunodermatology, ARUP Laboratories

References

Additional Resources