Background:

Intravenous immunoglobulin (IVIG) was originally licensed as antibody replacement therapy in patients with primary immunodeficiencies. Subsequent experimental use of IVIG during the last several decades, however, has shown that it is effective in numerous medical conditions. Currently there are six United States, Food and Drug Administration approved clinical indications for IVIG including: the treatment of primary immunodeficiencies, the prevention of bacterial infections in patients with hypogammaglobulinemia caused by B-cell chronic lymphocytic leukemia, the prevention of coronary artery aneurysms in Kawasaki disease, the prevention of infections, pneumonitis, and acute graft-versus-host disease after bone marrow transplantation, the reduction of serious bacterial infections in children with HIV, and the increase of platelet counts in patients with idiopathic thrombocytopenic purpura. IVIG treatment have been reported in the following dermatologic diseases: autoimmune blistering diseases, toxic epidermal necrolysis, Stevens Johnson syndrome, drug-induced hypersensitivity syndrome, pyoderma gangrenosum, pityriasis rubra pilaris, atopic dermatitis, dermatomyositis, scleromyxedema, nephrogenic fibrosing dermopathy, vasculitis, lupus erythematosus, psoriasis, polymorphous light eruption, urticaria, Behçet’s disease, scleroderma, Mucha-Habermann disease, hidradenitis suppurativa, acne vulgaris, streptococcal and staphylococcal toxic shock syndrome. The vast majority of these reports are in the form of individual case reports and small case series. A growing number of published reports suggest that IVIG treatment may be effective in the treatment of numerous inflammatory skin disease outside their currently approved indications. The following article provides a summary of the salient points in relation to the clinical use of intravenous immunoglobulin in dermatology.

Keywords: Intravenous immunoglobulin, off-label, treatment