Linear IgA Bullous Dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by linear deposits of IgA at the basement membrane zone on immunopathology. Direct immunofluorescence (DIF) remains the gold standard for diagnosis. Dapsone is the most commonly used therapeutic agent, but potential side effects such as hemolysis, agranulocytosis and methemoglobinemia in G6PD deficient patients necessitate the use of other treatments such as topical corticosteroids, tetracyclines, erythromycin, sulfonamides, nicotinamide, rituximab, omalizumab, methotrexate, cyclosporine and intravenous immunoglobulin.

We present a 7 year old Filipino female presenting with a 19 day history of multiple erythematous pruritic papules on the lower extremities which progressed to vesicles and bullae. Laboratory tests showed increased WBC, Koch’s Infection on Chest X-ray and a low G6PD Enzyme Assay. Skin punch biopsy and DIF also showed subepidermal blistering disease and findings consistent with LABD. The patient was placed on Prednisone 20 mg/5 mL syrup 5 mL for 2 weeks, Erythromycin 250 mg/5 mL syrup and Niacinamide 300 mg/paper tab for 1 month. She was also treated with Clobetasol Propionate 0.05% ointment and Betamethasone Valerate 0.1% ointment OD for 1 month. On follow up, the patient was well with no new onset of vesicles and bullae.

LABD has relied on Direct Immunofluorescence (DIF) for diagnosis and Dapsone as its first line treatment. Given that the patient has a low G6PD result, Dapsone was not given. Instead, systemic antibiotic and corticosteroids, Niacinamide supplement and topical corticosteroids was used as treatment. Patient factors such as age, comorbidities and disease severity play a role in therapeutic selection.

Human ; Female ; Child: 6-12 Yrs Old