Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: An update

Abstract

High-dose intravenous immunoglobulin (IVIg) is being increasingly utilized as an off-label therapy for a variety of autoimmune and inflammatory conditions across various specialties. Numerous reports have shown that it is an effective treatment for autoimmune skin blistering disorders. Unlike most therapies for blistering disorders, IVIg is not immunosuppressive and has a favorable side effect profile. This has allowed its use to expand dramatically over the last decade. However, due to the rarity and severity of autoimmune skin blistering diseases, well-designed prospective trials are generally lacking. This work highlights major research developments and the best evidence to date regarding the treatment of autoimmune pemphigus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, pemphigoid gestationis, and linear IgA dermatosis with IVIg, providing an update on its efficacy, proposed mechanisms of action, side effect profile, and indications for use.

Beneficial effect of a multimerized immunoglobulin Fc in an animal model of inflammatory neuropathy (experimental autoimmune neuritis)

“…In addition to high cost, IVIg has been associated with unwanted side effects, including headache, chills, migraine, dizziness, fever, nausea/vomiting, fatigue, and dermatitis (Bertorini et al ., ; Czernik et al ., ) . Moreover, renal failure, thromboembolic events, and anaphylaxis after IVIg treatment, although relatively rare, warrant consideration (Bertorini et al ., ; Czernik et al ., ) . Thus, developing an alternative for IVIg therapy in inflammatory neuropathies is desirable.…”

Pemphigoid gestationis successfully treated with intravenous immunoglobulin

“…Topical and oral corticosteroids are the mainstay of therapy,6 but when the disease persists, therapeutic options are limited 7 8. IVIG has been used to treat autoimmune skin blistering disorders, with successful suppression of blisters, allowing at the same time a reduction in the dose of corticosteroids 9. Given this, some authors reported the use of IVIG for treating PG, and it has occasionally been used in combination with immunosuppressants such as ciclosporin or azathioprine 10–15…”

Haemolytic Anaemia Following High Dose Intravenous Immunoglobulin Treatment for Epidermolysis Bullosa Acquisita

“…Although there are no randomised controlled trials of IVIg in EBA, numerous case series and reports have demonstrated good results [2] [3]. The incidence of clinically significant haemolytic anaemia post IVIg is relatively rare [4].…”