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Bullous Pemphigoid 230 Vesiculobullous Disorders

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Detects BP230 antibodies.

₹4,736₹6,766

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Bullous Pemphigoid 230 Vesiculobullous Disorders

  • Why is it done?
    • Detects circulating IgG autoantibodies against BP180 (collagen XVII), a hemidesmosomal protein located at the dermal-epidermal junction
    • Diagnose bullous pemphigoid (BP), an autoimmune blistering disorder characterized by subepidermal blisters and urticarial or eczematous lesions
    • Assist in differential diagnosis of other vesiculobullous disorders such as pemphigus vulgaris, dermatitis herpetiformis, and linear IgA disease
    • Evaluate patients presenting with persistent blistering, urticaria, or erosions with clinical suspicion of autoimmune blistering disease
    • Monitor disease activity and treatment response in confirmed bullous pemphigoid cases
    • Ordered when clinical presentation and skin biopsy findings suggest BP; typically performed during active disease when antibody titers are highest
  • Normal Range
    • Negative Result: < 0.9 U/mL or absent antibodies (laboratory-specific thresholds may vary); indicates absence of BP180-specific autoantibodies
    • Borderline/Equivocal Result: 0.9-1.1 U/mL (varies by assay); warrants repeat testing or additional confirmatory studies
    • Positive Result: > 1.1 U/mL; indicates presence of BP180-specific IgG autoantibodies consistent with bullous pemphigoid
    • Units: U/mL (units per milliliter); quantitative results provide information about antibody concentration
    • Interpretation: Negative results help exclude BP; positive results support BP diagnosis but should be correlated with clinical findings and skin biopsy with immunofluorescence. Reference ranges may vary between laboratories using different assay methodologies (ELISA, chemiluminescence, immunofluorescence).
  • Interpretation
    • Positive BP180 Antibodies (> 1.1 U/mL): Strongly suggests bullous pemphigoid diagnosis, especially when combined with compatible clinical presentation (tense blisters, urticarial plaques) and skin biopsy showing subepidermal blistering with IgG and C3 deposits along the basement membrane zone on direct immunofluorescence
    • Negative BP180 Antibodies (< 0.9 U/mL): Makes BP diagnosis less likely but does not completely exclude it; approximately 10-20% of BP cases (seronegative BP) lack detectable BP180 antibodies; consider testing for BP230 (BPAG3) antibodies or checking for early disease when antibody titers may be low
    • High Positive Values (> 10 U/mL): Indicate active disease with ongoing autoimmune response; correlation with clinical severity is generally good; values may decrease with effective treatment (corticosteroids, steroid-sparing agents)
    • Low Positive Values (1.1-3 U/mL): May occur in early disease, partially treated BP, or seronegative BP; requires clinical correlation and possibly repeat testing
    • Factors Affecting Results: Disease activity and duration; immunosuppressive therapy (may reduce antibody titers); timing of blood draw relative to disease onset; concurrent autoimmune conditions; assay methodology and laboratory-specific cutoff values; recent skin biopsies or procedures may affect results
    • Clinical Significance: BP180 antibodies are more commonly found in generalized BP (present in 60-90% of cases) compared to localized forms; their presence helps confirm diagnosis and guides treatment decisions; some evidence suggests antibody titers may correlate with disease activity and prognosis; useful for distinguishing BP from other autoimmune blistering diseases like pemphigus vulgaris (anti-desmoglein antibodies) or dermatitis herpetiformis (tissue transglutaminase antibodies)
  • Associated Organs
    • Primary Organ System: Integumentary system (skin); however, BP180 antigen is found in multiple locations including basement membranes throughout the body
    • Mucosal Involvement: Oral mucosa affected in 10-30% of BP cases; rarely involves esophagus, nasopharynx, larynx, and ocular conjunctiva; BP180 antibodies correlate with mucosal manifestations
    • Diseases Associated with Abnormal Results: Bullous pemphigoid; bullous pemphigoid variants (pemphigoid gestationis, cicatricial pemphigoid); linear IgA disease (though primarily BP230 target); seronegative bullous diseases; BP may overlap with other autoimmune conditions
    • Associated Malignancies: Paraneoplastic pemphigoid (anti-BP180) linked to various cancers including gastric, lung, breast, ovarian, and lymphoid malignancies; screening for underlying malignancy may be warranted particularly in older patients with new-onset BP
    • Complications and Risks: Secondary skin infections due to blistering and erosions; sepsis (particularly in elderly patients); severe cases may affect mucous membranes; scarring and milia formation; psychological impact from chronic disfiguring skin lesions; complications from long-term corticosteroid treatment (immunosuppression, metabolic effects, infections); mortality risk primarily related to infection and corticosteroid toxicity in elderly patients
  • Follow-up Tests
    • BP230 (BPAG3) Antibody Testing: Recommended if BP180 is negative or borderline; approximately 10-40% of BP cases show isolated BP230 positivity; helps identify seronegative BP cases
    • Skin Biopsy with Direct Immunofluorescence (DIF): Gold standard for confirming BP diagnosis; shows IgG and C3 deposits along basement membrane zone; should be performed from perilesional or lesional skin on normal-appearing skin; helps confirm diagnosis when serology is negative or uncertain
    • Skin Biopsy with Histopathology: Reveals subepidermal blister formation with intact basal layer ("floor of the blister"), eosinophil-rich infiltrate, and differentiation from other bullous disorders
    • Indirect Immunofluorescence (IIF) or Immunoblotting: Additional confirmatory tests using patient serum; alternative to ELISA for detecting circulating autoantibodies; helps define antibody target (BP180 vs BP230)
    • Pemphigus Antibody Panel (Anti-desmoglein 1 and 3): Performed if clinical presentation is ambiguous; helps differentiate BP from pemphigus vulgaris or pemphigus foliaceus, which present with intraepidermal rather than subepidermal blistering
    • Tissue Transglutaminase (tTG) IgA: If dermatitis herpetiformis is suspected; helps rule out gluten-sensitive enteropathy-associated blistering disorder
    • Malignancy Screening: Age-appropriate cancer screening (chest X-ray, colonoscopy, serum markers); particularly important if paraneoplastic pemphigoid suspected, in elderly patients with new-onset BP, or if clinical features atypical
    • Monitoring Frequency for Ongoing Conditions: Repeat BP180 antibody testing every 3-6 months during active disease to assess treatment response; more frequent monitoring during therapy initiation or dose changes; less frequent testing once disease is controlled; some patients with clinical remission may retain low-level positive serology
    • Complete Blood Count and Chemistry Panel: Monitor for corticosteroid side effects, electrolyte abnormalities, and complications from immunosuppressive therapy
  • Fasting Required?
    • Fasting Requirement: No
    • Food and Drink: Fasting is not required; patient may eat and drink normally prior to blood draw
    • Medications to Avoid: No specific medications need to be discontinued; however, inform physician of all current medications including corticosteroids, immunosuppressive agents, biologics (dupilumab for atopic dermatitis may affect results), and anticoagulants for procedural planning; antibody results should be interpreted in context of current treatment regimen
    • Patient Preparation Requirements: Inform phlebotomist about bleeding disorders or anticoagulation therapy; notify lab staff of recent blood transfusions (within 6 months) as these may affect test results; avoid strenuous exercise immediately before blood draw; wear loose, comfortable clothing to facilitate venipuncture; timing of draw not critical but same time of day preferably for serial monitoring to reduce variation
    • Sample Collection: Blood sample collected via venipuncture into serum separator tube (SST) or similar collection tube as per laboratory protocol; minimal volume required (typically 5-10 mL); sample should be labeled with patient identification and sent to laboratory promptly for processing and testing

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