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Desmoglein III Antibody
Immunity
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No Fasting Required
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Autoantibodies against Desmoglein III are highly specific for pemphigus vulgaris, a serious autoimmune blistering disorder affecting the skin and mucous membranes
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Desmoglein III Antibody Test Information Guide
- Why is it done?
- Detects autoantibodies against desmoglein III, a protein component of desmosomes that provides cell-to-cell adhesion in the epidermis and mucous membranes
- Diagnosis of mucosal pemphigus vulgaris (anti-Dsg3 antibodies alone) and mucocutaneous pemphigus vulgaris (anti-Dsg3 and anti-Dsg1 antibodies together)
- Assessment of disease severity and prognosis in pemphigus vulgaris patients
- Monitoring disease activity and response to treatment in pemphigus vulgaris
- Screening for anti-Dsg3 antibodies in patients with chronic oral ulcers, blisters, or erosions
- Performed when pemphigus vulgaris is clinically suspected and initial serological screening is positive
- Normal Range
- Negative Result: < 0.9 U/mL or negative; indicates absence of anti-Dsg3 antibodies
- Borderline Result: 0.9-1.0 U/mL; uncertain result requiring correlation with clinical findings and repeat testing
- Positive Result: > 1.0 U/mL; indicates presence of anti-Dsg3 antibodies; higher titers correlate with increased disease severity
- Measurement Units: U/mL (Units per milliliter) or mmol/L depending on laboratory methodology; ELISA (enzyme-linked immunosorbent assay) is the standard method
- Normal vs. Abnormal: Negative or undetectable levels are normal; any positive result indicates autoimmune antibody formation against Dsg3 and warrants clinical correlation and further investigation for pemphigus vulgaris or related autoimmune blistering disorders
- Interpretation
- Negative Anti-Dsg3 Result: Excludes mucosal-dominant pemphigus vulgaris; patient unlikely to have pemphigus vulgaris if clinical presentation and other serology are consistent
- Isolated Anti-Dsg3 Positivity (Anti-Dsg1 Negative): Indicates mucosal-dominant or anti-Dsg3-only pemphigus vulgaris; primarily affects oral mucosa and potentially pharyngeal/laryngeal tissues; generally more favorable prognosis than mucocutaneous disease
- Anti-Dsg3 and Anti-Dsg1 Double Positivity: Diagnostic of mucocutaneous pemphigus vulgaris; indicates more severe disease with involvement of both oral mucosa and cutaneous surfaces; associated with greater disease burden and potential systemic complications
- High Antibody Titers: Correlate with disease activity, severity, and extent of lesions; higher titers often indicate active disease requiring more aggressive immunosuppressive therapy
- Decreasing Antibody Titers: Indicate favorable response to immunosuppressive treatment; may precede clinical remission by weeks to months; useful for monitoring treatment efficacy
- Persistently High or Rising Titers: Suggest inadequate disease control or impending clinical relapse; may warrant adjustment of immunosuppressive therapy or escalation of treatment
- Factors Affecting Results: Timing of blood draw relative to disease activity, current immunosuppressive therapy, individual immune response variation, presence of other autoimmune conditions, laboratory methodology differences, and sample handling or storage issues
- Associated Organs
- Primary Organ System: Integumentary (skin) and mucosal epithelial tissues; affects epidermis, mucous membranes of oral cavity, pharynx, larynx, and potentially esophagus
- Pemphigus Vulgaris: Most common autoimmune blistering disorder associated with anti-Dsg3 antibodies; characterized by intraepidermal blistering (acantholysis) due to loss of cell adhesion
- Mucosal-Dominant Pemphigus Vulgaris: Anti-Dsg3 alone; involves primarily oral mucosa with chronic erosions, ulcers, and difficulty eating or speaking; affects approximately 50-70% of pemphigus vulgaris patients
- Mucocutaneous Pemphigus Vulgaris: Anti-Dsg3 and anti-Dsg1 antibodies present; involves both mucosal and cutaneous sites; more severe with widespread skin involvement and systemic complications
- Associated Complications: Secondary bacterial infections of erosions, nutritional compromise from oral ulcers, laryngeal involvement causing dysphonia or aspiration risk, esophageal involvement with dysphagia, psychological impact from facial blistering and scarring, and potential involvement of respiratory or gastrointestinal tracts in severe cases
- Related Autoimmune Blistering Disorders: Paraneoplastic pemphigus, pemphigus foliaceus variants, and occasionally anti-Dsg3 antibodies may be detected in other mucosa-predominant pemphigus variants requiring differentiation based on clinical presentation and complementary testing
- Follow-up Tests
- Desmoglein I (Dsg1) Antibody Test: Essential complementary test to determine if anti-Dsg1 antibodies are also present; distinguishes mucosal-dominant from mucocutaneous disease; should be performed simultaneously or immediately after Dsg3 testing
- Skin Biopsy with Direct Immunofluorescence (DIF): Gold standard for confirming pemphigus diagnosis; demonstrates IgG and C3 deposition in an intercellular pattern; recommended if serology is positive or clinical suspicion remains high despite negative antibody testing
- Oral or Cutaneous Biopsy with Histopathology: Confirms acantholysis and intraepidermal blistering pattern; distinguishes pemphigus vulgaris from other blistering disorders; recommended if diagnosis remains uncertain after serology
- Indirect Immunofluorescence (IIF) Serology: Provides qualitative assessment of circulating IgG antibodies and confirms their intercellular pattern; may be used as initial screening or confirmatory test
- Immunofluorescence Microscopy on Patient Serum: Detects circulating IgG antibodies to identify any uncharacterized antibodies or variants not detected by routine ELISA testing
- Serial Antibody Titer Monitoring: Repeat Dsg3 antibody testing every 3-6 months during active treatment to assess therapeutic response and monitor for disease relapse; frequency may be adjusted based on initial titer and clinical status
- Complete Blood Count (CBC) and Comprehensive Metabolic Panel (CMP): Baseline laboratory studies before initiating immunosuppressive therapy; monitor for medication-related adverse effects during treatment
- Malignancy Screening: Age-appropriate cancer screening as pemphigus vulgaris can rarely occur as paraneoplastic syndrome; particularly consider if atypical clinical presentation or concurrent malignancy risk factors present
- Additional Autoimmune Antibodies: Antinuclear antibodies (ANA) or other autoimmune markers if clinical presentation suggests concurrent autoimmune disease or atypical pemphigus variant
- Fasting Required?
- Fasting Required: NO - No fasting is required for the Desmoglein III Antibody test
- Sample Collection: Standard venipuncture collection into serum separator tube (SST) or appropriate tube per laboratory protocol; can be drawn at any time of day without dietary restrictions
- Medications to Avoid: No medications need to be held or avoided before test collection; however, document current immunosuppressive therapy (corticosteroids, azathioprine, mycophenolate mofetil, rituximab, etc.) as it may influence interpretation of antibody titers
- Patient Preparation: Patient should notify healthcare provider of any recent infections, immunizations, or changes in medications; inform phlebotomist if anticoagulants are being used or if patient has bleeding disorders
- Specimen Handling: Blood specimen should be allowed to clot at room temperature for 30 minutes before centrifugation; serum should be separated and refrigerated at 2-8°C or frozen at -20°C until transport to laboratory; avoid hemolysis and ensure proper tube labeling with patient identifiers and collection time
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