Desmoglein I Antibody

Immunity

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Details

These autoantibodies are associated with autoimmune blistering skin diseases, primarily pemphigus foliaceus.

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Desmoglein I Antibody Test Information Guide

Why is it done?
- Detects autoimmune antibodies against desmoglein I, a cell adhesion protein found in the epidermis
- Diagnoses pemphigus vulgaris and pemphigus foliaceus, autoimmune blistering skin diseases
- Monitors disease activity and treatment response in pemphigus patients
- Assists in differentiating between different types of pemphigus and other autoimmune blistering disorders
- Typically performed when patients present with intraepidermal blistering, mucosal ulceration, or characteristic flaccid blisters
Normal Range
- Negative (Undetectable): <0.9 U/mL or negative result indicates absence of anti-desmoglein I antibodies
- Positive: ≥1.0 U/mL or positive result suggests presence of anti-desmoglein I antibodies
- Units: U/mL (units per milliliter) - measured by indirect immunofluorescence or ELISA methodology
- Normal result: Negative indicates no autoimmune antibodies against desmoglein I; patient unlikely to have pemphigus
- Abnormal result: Positive indicates presence of anti-desmoglein I antibodies; consistent with pemphigus diagnosis
- Reference ranges may vary slightly between laboratories based on assay methodology
Interpretation
- Negative Result (<0.9 U/mL): Suggests absence of pemphigus; however, early disease or clinical remission may show negative results despite symptoms; additional testing or clinical correlation recommended
- Positive Result (≥1.0 U/mL): Highly suggestive of pemphigus vulgaris or pemphigus foliaceus; correlation with clinical presentation and other serological markers (desmoglein III) necessary for definitive diagnosis
- Desmoglein I-Only Positive: Typically indicates pemphigus foliaceus (superficial blistering); primarily affects skin with mucosal surfaces usually spared
- Desmoglein I and III Both Positive: Indicates pemphigus vulgaris with both mucosal and cutaneous involvement; more severe disease pattern
- Titer Levels: Higher antibody levels generally correlate with disease activity; rising titers suggest disease flare; declining titers indicate therapeutic response to treatment
- Factors Affecting Results: Immunosuppressive therapy (systemic corticosteroids, biologics) may lower antibody titers; disease duration; treatment compliance; laboratory methodology variations; timing of blood draw relative to flare cycles
- Clinical Significance: Anti-desmoglein I antibodies are pathogenic, directly causing acantholysis (loss of cell-to-cell adhesion); result is one component of comprehensive diagnostic assessment including skin biopsy with immunofluorescence
Associated Organs
- Primary Organ System: Integumentary system (skin); mucous membranes (oropharyngeal, conjunctival)
- Pemphigus Foliaceus: Superficial epidermal blistering at granular layer; primarily cutaneous manifestations; typically spares oral mucosa
- Pemphigus Vulgaris: Suprabasal blistering; affects both skin and mucous membranes; oral involvement common and often first presentation; esophageal involvement possible
- Associated Diseases and Conditions: Autoimmune pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus (with underlying malignancy), drug-induced pemphigus
- Potential Complications: Severe blistering and erosions; secondary bacterial infections; malnutrition from oral involvement; dehydration from extensive skin loss; esophageal strictures; sepsis if untreated; psychological impact from chronic disfiguring condition
- Disease Severity Relationship: Antibody titer correlates with disease extent; higher titers associated with more extensive mucocutaneous involvement
Follow-up Tests
- Desmoglein III Antibody Testing: Distinguishes pemphigus vulgaris from pemphigus foliaceus; anti-Dsg3 positivity indicates mucosal involvement potential
- Direct Immunofluorescence (DIF) on Skin Biopsy: Gold standard for pemphigus diagnosis; shows intercellular IgG and complement deposition in epidermis
- Indirect Immunofluorescence (IIF) Microscopy: Detects circulating antibodies; provides semiquantitative assessment through serum dilution titers
- Histopathology (H&E Staining): Demonstrates acantholysis pattern; confirms intraepidermal blistering; evaluates biopsy specimen for disease confirmation
- Baseline Investigations if Positive Result: Malignancy screening (chest X-ray, CT imaging) for paraneoplastic pemphigus; complete blood count; metabolic panel; liver and renal function tests
- Monitoring During Treatment: Serial anti-desmoglein I antibody titers every 3-6 months during active disease; repeat testing at baseline, during therapy, and at clinical remission to monitor therapeutic response; antibody titers decline with successful immunosuppressive therapy
- Complementary Serological Tests: Envoplakin and periplakin antibodies (for paraneoplastic pemphigus); BP180 and BP230 antibodies (to exclude bullous pemphigoid)
- Long-term Follow-up Frequency: Initial diagnosis: testing interval 2-4 weeks; during active treatment: monthly or bimonthly; after achieving remission: every 3-6 months; for prevention of relapse and dose adjustment of immunosuppressive medications
Fasting Required?
- Fasting Requirement: No - Fasting is not required for desmoglein I antibody testing
- Blood Collection Timing: Test can be performed at any time of day; no specific time restrictions; random blood draw acceptable
- Specimen Collection: 5-10 mL serum or plasma in standard serum separator tube (SST); room temperature collection acceptable; no special preservation requirements
- Medications to Avoid: No medications need to be withheld for this test; immunosuppressive medications (corticosteroids, azathioprine, mycophenolate) do not interfere with test accuracy; continue all regularly prescribed medications
- Patient Preparation: No special preparation necessary; patient may eat and drink normally; no activity restrictions; wear comfortable clothing to facilitate venipuncture
- Pre-test Considerations: Stay well-hydrated to facilitate blood draw; inform phlebotomist of current medications if taking anticoagulants; report any bleeding disorders or medications affecting coagulation; note timing of last dose of immunosuppressive therapy if monitoring disease control
- Specimen Handling: Serum separated within 2 hours of collection; samples can be refrigerated at 2-8°C if not immediately tested; frozen samples stable for extended periods; proper labeling and chain of custody essential

How our test process works!