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    Subjects/Dermatology/Linear IgA Bullous Disease
    Linear IgA Bullous Disease
    medium
    hand Dermatology

    A 64-year-old woman with MRSA endocarditis develops a tense bullous eruption on day 8 of intravenous vancomycin therapy. Examination reveals tense, fluid-filled bullae arranged in characteristic annular and polycyclic plaques with new lesions forming at the periphery of older lesions — producing a "string of pearls" appearance. Lesions favor the trunk, buttocks, and groin, with mucosal involvement of the oral cavity. Punch biopsy shows a subepidermal blister with neutrophil-predominant infiltrate. Direct immunofluorescence of perilesional skin is shown in the diagram at **B**. Which of the following is the most appropriate next step in management?

    A. Discontinue vancomycin immediately and initiate dapsone after G6PD screening
    B. Continue vancomycin with topical corticosteroids only
    C. Switch to cephalosporin-based antibiotic therapy with systemic corticosteroids
    D. Perform HLA-DQ2/DQ8 testing and initiate a gluten-free diet

    Explanation

    Why "Discontinue vancomycin immediately and initiate dapsone after G6PD screening" is right

    The structure marked B shows linear IgA deposition along the basement membrane zone in a characteristic "string of pearls" pattern — pathognomonic for Linear IgA Bullous Disease (LABD). In the adult setting, vancomycin is the most common and best-characterized drug trigger for LABD. The clinical presentation (tense bullae, annular/polycyclic arrangement, mucosal involvement, neutrophil-predominant subepidermal blister, and linear IgA on DIF) confirms drug-induced LABD. Management mandates immediate withdrawal of the offending agent; drug-induced disease typically resolves 2–6 weeks after discontinuation. First-line systemic therapy is dapsone (after mandatory G6PD screening to exclude deficiency and risk of hemolysis), which controls disease in days. [British Association of Dermatologists Autoimmune Bullous Disease Guidelines; Bolognia 5e]

    Why each distractor is wrong

    • Continue vancomycin with topical corticosteroids only: Continuing the offending drug perpetuates the autoimmune response and prevents resolution. Topical therapy alone is insufficient for extensive mucosal and cutaneous disease. The anchor diagnosis requires drug withdrawal.
    • Switch to cephalosporin-based antibiotic therapy with systemic corticosteroids: Although cephalosporins are an alternative for MRSA coverage, ceftriaxone itself is a known trigger of LABD in some cases. Systemic corticosteroids are not first-line for LABD; dapsone is superior and acts more rapidly.
    • Perform HLA-DQ2/DQ8 testing and initiate a gluten-free diet: This approach is appropriate for dermatitis herpetiformis (DH), which presents with granular IgA in dermal papillae and is associated with celiac disease. The linear IgA along the BMZ at B excludes DH and confirms LABD, which is not gluten-related.
    High-YieldNEET PG
    Vancomycin-induced LABD in adults: withdraw drug + dapsone (after G6PD screen) = rapid resolution; linear IgA at BMZ distinguishes LABD from granular IgA in dermal papillae of DH.

    [British Association of Dermatologists Autoimmune Bullous Disease Guidelines; Bolognia 5e]

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