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Bullous Pemphigoid 180 Vesiculobullous Disorders

Immunity
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Report in 192Hrs

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Autoantibody test against BP180 antigen.

₹4,736₹6,766

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Bullous Pemphigoid 180 Vesiculobullous Disorders - Comprehensive Test Guide

  • Section 1: Why is it done?
    • Test Description: This test detects circulating IgG antibodies directed against BP180 (collagen XVII), a transmembrane protein located at the dermal-epidermal junction. These autoantibodies are pathognomonic for bullous pemphigoid, a chronic autoimmune blistering disorder.
    • Primary Indications: Diagnosis of suspected bullous pemphigoid in patients presenting with tense blisters and urticarial lesions
    • Confirmation of atypical or seronegative pemphigoid presentations
    • Differentiation from pemphigus vulgaris and other vesiculobullous disorders
    • Monitoring disease activity and treatment response in established bullous pemphigoid
    • Screening for medication-induced bullous pemphigoid (e.g., from dipeptidyl peptidase-4 inhibitors)
    • Typical Timing: Performed at initial clinical presentation with suspected bullous pemphigoid, prior to or concurrent with skin biopsy for histopathological confirmation. Repeated serum testing may be ordered during follow-up to assess treatment efficacy and disease remission.
  • Section 2: Normal Range
    • Reference Values: Negative: <0.9 U/mL (units may vary by laboratory; typical cutoff ranges from 0.8-1.0 U/mL)
    • Borderline/Equivocal: 0.9-1.1 U/mL (may require repeat testing or clinical correlation)
    • Positive: >1.1 U/mL (highly suggestive of bullous pemphigoid; values often significantly elevated in active disease)
    • Units of Measurement: U/mL (units per milliliter) using ELISA methodology; some laboratories may report as Index Values or qualitative results (Negative/Positive)
    • Interpretation: Negative result indicates absence of detectable BP180 antibodies, making bullous pemphigoid unlikely (though 10-15% of pemphigoid patients may be seronegative). Positive result strongly supports the diagnosis of bullous pemphigoid when combined with compatible clinical and histological findings. Higher antibody titers often correlate with greater disease activity.
  • Section 3: Interpretation
    • Positive Result (>1.1 U/mL): Strongly indicates active bullous pemphigoid, particularly in patients with clinical signs of tense blisters, urticarial plaques, or mucosal involvement. Antibody levels often correlate with disease activity; higher titers suggest more severe or widespread disease. False positives are rare (<2% specificity), making this a highly specific test.
    • Negative Result (<0.9 U/mL): Does not exclude bullous pemphigoid diagnosis. Approximately 10-15% of confirmed pemphigoid patients are BP180-seronegative. These patients may have antibodies against BP230 (a 230-kDa antigen) instead, or represent the rare mucosal-dominant form. Clinical and histopathological correlation is essential.
    • Borderline Result (0.9-1.1 U/mL): Requires clinical correlation with patient presentation and biopsy findings. Repeat testing after 1-2 weeks may clarify results. Consider testing for BP230 antibodies and performing direct immunofluorescence on skin biopsy.
    • Factors Affecting Results: Early disease stage may show negative serology despite clinical symptoms; Immunosuppressive therapy (corticosteroids, azathioprine) may reduce antibody titers; Specimen hemolysis or improper handling; Laboratory variation in assay sensitivity; Patient genetics and immune response variability; Concurrent medications affecting immune function.
    • Clinical Significance Patterns: Positive BP180 antibodies with direct immunofluorescence showing linear IgG and C3 deposits = Definitive bullous pemphigoid diagnosis; Positive BP180 with negative BP230 = More typical pemphigoid pattern; High titers with clinical improvement during treatment = Favorable prognostic indicator; Persistent elevated titers despite treatment = May indicate inadequate disease control or need for treatment adjustment.
  • Section 4: Associated Organs
    • Primary Organ System: Integumentary system (skin and mucous membranes); Specifically targets the dermal-epidermal junction through hemidesmosomes.
    • Secondary Sites of Involvement: Oral mucosa (15-34% of patients) with blisters, erosions, and painful ulcerations; Conjunctival and ocular mucosa (leading to cicatricial complications if severe); Pharyngeal and laryngeal involvement causing dysphagia or airway compromise (rare); Genital and perianal mucosa (in extensive disease); Esophageal involvement causing strictures (uncommon but serious).
    • Associated Medical Conditions: Diabetes mellitus (particularly type 2, 1.6-2.5 times increased risk); Hypertension; Dermatitis herpetiformis; Autoimmune thyroid disease; Pernicious anemia; Systemic lupus erythematosus; Rheumatoid arthritis; Medication-induced (DPP-4 inhibitors, diuretics, penicillamine, ACE inhibitors).
    • Potential Complications: Secondary bacterial infections of ruptured blisters (cellulitis, abscess formation); Systemic infection/sepsis if widespread; Scarring and post-inflammatory hyperpigmentation; Ocular cicatricial changes leading to blindness if untreated; Esophageal strictures limiting oral intake; Psychological distress and impaired quality of life; Complications from prolonged immunosuppressive therapy (infections, malignancy risk); Steroid-induced side effects (diabetes, osteoporosis, hypertension).
    • Immune System Impact: Th2-mediated autoimmune response; Elevated IgG and IgE antibodies; Complement activation leading to tissue damage; T-cell abnormalities and reduced regulatory T cells.
  • Section 5: Follow-up Tests
    • Confirmatory Diagnostic Tests: Skin biopsy from perilesional area for histopathology (shows subepidermal blistering with preserved basal layer); Direct immunofluorescence (linear IgG and C3 at basement membrane); Indirect immunofluorescence on patient serum; BP230 antibody testing (if BP180 negative); Enzyme-linked immunosorbent assay (ELISA) for anti-BP180 NC16a domain.
    • Disease Activity Monitoring: Repeat BP180 antibody serology every 3-6 months during active treatment; Serial testing correlates with clinical response; Decreasing titers indicate treatment efficacy; Persistently elevated or rising titers may necessitate treatment adjustment.
    • Treatment Monitoring Tests: Complete blood count (if using azathioprine, methotrexate, or rituximab); Liver function tests (azathioprine, methotrexate monitoring); Renal function panel (general steroid and immunosuppressant assessment); Serum glucose (steroid-induced hyperglycemia); Bone mineral density/DEXA scan (prolonged corticosteroid use); Tuberculosis screening (before biologic/immunosuppressant therapy).
    • Differential Diagnosis Tests: Desmoglein 1 and 3 antibodies (pemphigus vulgaris exclusion); Linear IgA disease serology; Dermatitis herpetiformis-associated tissue transglutaminase (tTG) antibodies; Anti-desmoglein testing panel.
    • Recommended Monitoring Frequency: Initial diagnosis: BP180 testing, biopsy, and direct immunofluorescence simultaneously; During active treatment: Serology every 6-8 weeks for first 3 months, then every 3 months; During remission maintenance: Every 6-12 months; Baseline and annual monitoring: CBC, liver/renal function if on immunosuppressants; Post-treatment follow-up: 6-12 months after achieving remission to assess relapse risk.
    • Complementary Diagnostic Tests: Antinuclear antibody (ANA) and anti-tissue specific antibody panel (associated autoimmune diseases); Thyroid function tests (TSH, free T4); Celiac serologies (tTG-IgA, endomysial antibodies); Infectious disease screening (hepatitis B/C, HIV, TB) before immunosuppression.
  • Section 6: Fasting Required?
    • Fasting Status: NO - Fasting is not required for BP180 antibody serology testing.
    • Sample Collection Requirements: Blood sample collection via venipuncture into standard serum separator tube (SST) or EDTA tube depending on laboratory protocol; Typically 5-10 mL of blood needed; Can be collected any time of day regardless of meal intake; No special preparation or abstinence required.
    • Pre-Test Instructions: No food or drink restrictions; Continue all regular medications unless otherwise instructed; Stay hydrated (improves venipuncture); Avoid excessive activity immediately before collection; Inform phlebotomist of any bleeding disorders or anticoagulation therapy.
    • Medications - No Special Precautions: Continue topical corticosteroids (does not affect serum antibody levels); Continue systemic corticosteroids (may reduce antibody titers but not contraindicated); Continue immunosuppressive medications (azathioprine, methotrexate, mycophenolate, rituximab); Continue anticoagulants if on warfarin or DOACs (inform phlebotomist); Antihistamines do not interfere with testing.
    • Sample Handling & Timing: Serum must be separated within 1-2 hours of collection; Refrigerate separated serum at 2-8°C if testing cannot be performed same day; Freeze at -20°C or lower for long-term storage; Avoid repeated freeze-thaw cycles; Send to laboratory promptly (stable for 3-7 days refrigerated).
    • Additional Patient Preparation: Skin biopsy sites (if being performed) should not be sampled immediately from new lesions treated with topical medications; Allow 24-48 hours after topical application for clearer histological visualization; Document current treatment medications and dosages; Note duration of illness and symptom timeline; Photograph clinical lesions if possible for correlation with serology results.

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