Dilemmatic presentation of linear IgA disease: a case report

Linear IgA disease is a rare blistering disease affecting the skin and less often the oral mucosa. This disease has a varied clinical presentation especially when the disease affects the oral mucosa. Certain drugs, autoimmune diseases, infection, chronic renal insufficiency, and malignancies have been implicated as the etiologic factors of this disease. The gold standard for diagnosis of linear IgA disease is direct immunofluorescence on fresh tissue. The immunofluorescence will demonstrate a homogeneous linear deposition of immunoglobulin A along the basement membrane. Multiple therapeutic agents, including corticosteroids, dapsone, sulfapyridine, and colchicine (either alone or in combination), have been used. The authors report a case of linear IgA disease with ambiguous features of pemphigus and cicatricial pemphigoid.