A case of refractory linear IgA disease responsive to high-dose intravenous immunoglobulin therapy

Management of linear IgA disease (LAD) is a challenging task due to its rarity and lack of well-established treatment guidelines. We describe a case of refractory LAD that was induced into remission by high-dose intravenous immunoglobulin (IVIg) therapy. A 49-year-old woman was referred for a second opinion on the management of severe refractory LAD with cutaneous and mucosal involvement. Initial diagnosis was made based on clinical grounds, following two equivocal skin biopsies. Histopathological characteristics were finally identified on a biopsy collected during a disease flare. Therapeutic course was tumultuous, punctuated by side effects to numerous immunosuppressive agents. Her disease activity remained suboptimally controlled on mycophenolate mofetil 3 g daily and prednisolone 10 mg daily. Following a six-month trial of IVIg therapy (2g/kg), we were able to induce stable remission. She was successfully weaned off prednisolone and the daily mycophenolate dosage was reduced to 2 g. Our case illustrates the utility of high-dose IVIg therapy, which may be sufficient in inducing remission in the context of background maintenance immunosuppressants. While IVIg is not supported for use in LAD in Australia, we are hopeful that with increasing case reports and possibly controlled trials, there will be improved accessibility for managing refractory cases in the future.