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Dermatitis herpetiformis (DH) is a chronic, pruritic skin disease usually associated with gluten-sensitive enteropathy (GSE – celiac disease).
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 of complement (C3), and fibrinogen in biopsy specimens of perilesional skin (3 mm from fresh/actively inflamed lesion) from patients suspected of having dermatitis herpetiformis; perform this test with serum Use in testing for celiac disease (celiac reflexive panel), which is associated with dermatitis herpetiformis in most cases, along with pemphigoid and pemphigus panel tests 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; specimen must have epidermis/epithelium and basement membrane zone (BMZ) Granular and fibrillar IgA immune deposits may be sparse; use of more than one specimen increases detection of diagnostic findings 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 for IgA endomysial and tissue transglutaminase antibodies to make diagnosis and to follow 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: 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: May be negative if patient is following a gluten-free diet False-positive IgA antibody levels may occur in other inflammatory bowel diseases Do not use in IgA-deficient individuals; acceptable reflex screening test for celiac disease Celiac Disease Reflexive Panel or Tissue Transglutaminase (tTG) Antibody, IgA, assay is the preferred screening test |
| Follow-up: Use tissue transglutaminase (tTG) antibody, IgA with reflex to endomysial antibody, IgA by IFA for initial diagnosis and to follow disease activity in dermatitis herpetiformis Repeat test for indeterminate results and/or continuing clinical consideration of dermatitis herpetiformis 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 See Immunobullous Skin Diseases Testing algorithm |
| Limitations: Clinical correlation necessary because the incidence of false positives, although rare, increases with age Dermatitis herpetiformis may not be detected when parts of monkey esophagus substrate without smooth muscle (proximal 2/3) are used Because of clinical overlap among immunobullous diseases and similar names, pemphigoid testing may be confused with pemphigus testing and inadvertently misordered |
| 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: Useful for pemphigus immunobullous disease but likely will not detect dermatitis herpetiformis because test identifies IgG rather than the IgA antibodies that characterize dermatitis herpetiformis 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: Epithelial Skin Antibody |
| ARUP #: 0090299 |
| Methodology: Indirect Immunofluorescence (Indirect Fluorescent Antibody) |
| Use: Panel includes epithelial basement membrane zone (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 linear IgA disease, pemphigoid, epidermolysis bullosa acquisita, pemphigus, and dermatitis herpetiformis in patients suspected of having or known to have any type of subepidermal immunobullous disease Use along with tTG IgA with reflex to endomysial antibody for diagnosis of dermatitis herpetiformis |
| 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: Endomysial Antibody, IgA by IFA |
| ARUP #: 0050736 |
| Methodology: Semi-Quantitative Indirect Fluorescent Antibody |
| Use: Use along with tissue transglutaminase antibody testing to diagnose celiac disease in association with dermatitis herpetiformis Perilesional skin biopsy for DIF is helpful in diagnosis (>90% of dermatitis herpetiformis cases are positive) |
| Follow-up: Use endomysial antibody, IgA by IFA and/or tissue transglutaminase (tTG) antibody, IgA and/or epidermal transglutaminase (TGe) antibody, IgA tests to follow dermatitis herpetiformis disease activity unless IgA deficient or predominant IgG antibodies See Immunobullous Skin Diseases Testing algorithm |
| Test name: Celiac Disease Dual Antigen Screen with Reflex |
| ARUP #: 2002026 |
| Methodology: Semi-Quantitative Enzyme-Linked Immunosorbent Assay |
| Use: Diagnose celiac disease in association with suspected or known dermatitis herpetiformis Components include celiac disease dual antigen screen; tissue transglutaminase antibodies IgA and IgG; and gliadin peptide antibodies IgA and IgG Perilesional skin biopsy by DIF is helpful in diagnosis (>90% of dermatitis herpetiformis cases are positive) |
| Limitations: May be negative if patient is following a gluten-free diet Some patients with DH will also be negative |
| Follow-up: Use to establish diagnosis of celiac disease which is present in virtually all patients with dermatitis herpetiformis |
| Test name: Tissue Transglutaminase (tTG) Antibody, IgA |
| ARUP #: 0097709 |
| Methodology: Semi-Quantitative Enzyme-Linked Immunosorbent Assay |
| Comments: Not recommended as single test in evaluation of immunobullous skin disease Do not use in IgA deficient individuals Use to monitor disease activity May be positive in inflammatory bowel disease and other disorders than celiac disease and associated dermatitis herpetiformis Use tissue transglutaminase (tTG) antibody, IgA and/or endomysial antibody, IgA by IFA and/or epidermal transglutaminase (TGe) antibody, IgA tests to follow dermatitis herpetiformis disease activity unless IgA deficient or predominant IgG antibodies See Immunobullous Skin Diseases Testing algorithm |
| Test name: Tissue Transglutaminase Antibody, IgG |
| ARUP #: 0056009 |
| Methodology: Semi-Quantitative Enzyme-Linked Immunosorbent Assay |
| Comments: Not recommended as single test in evaluation of immunobullous skin disease Order in patients with suspected celiac disease and documented IgA deficiency May be used for monitoring If IgA deficient and/or IgG celiac disease antibodies are present, use tissue transglutaminase IgG and/or endomysial antibody, IgG to follow dermatitis herpetiformis disease activity See Immunobullous Skin Diseases Testing algorithm |
| Test name: Endomysial Antibody, IgG |
| ARUP #: 2005501 |
| Methodology: Semi-Quantitative Indirect Fluorescent Antibody |
| Comments: Use in IgA deficient individuals to evaluate for celiac disease associated with dermatitis herpetiformis If IgA deficient and/or IgG celiac disease antibodies are present, use tissue transglutaminase IgG and/or endomysial antibody, IgG to follow dermatitis herpetiformis disease activity See Immunobullous Skin Diseases Testing algorithm |