Intravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses

Abstract

Intravenous immunoglobulins are an effective and well-tolerated treatment option for immune dermatological diseases. However, they are primarily used to treat diseases with a severe course and are mostly used off-label. Therefore, it is important to document case series on the use of intravenous immunoglobulins in rare immune dermatological diseases. We present here 32 patients who were treated with intravenous immunoglobulins in our clinic between 2015 and 2020. The indications were dermatomyositis, including amyopathic and paraneoplastic forms, as well as overlap-syndromes (n = 18), pemphigus vulgaris (n = 2), mucous membrane pemphigoid (n = 2), linear IgA dermatosis (n = 1), necrotizing vasculitis (n = 1), urticarial vasculitis (n = 1), systemic scleroderma (n = 1), Stevens-Johnson syndrome (n = 1), pyoderma gangrenosum (n = 3), and livedoid vasculopathy (n = 2). The data from this case series confirm the efficacy and tolerability of intravenous immunoglobulins as an adjuvant treatment option for rare therapy-resistant immune dermatoses.

Keywords

• intravenous immunoglobulin
• dermatomyositis
• autoimmune bullous dermatosis
• vasculitis
• stevens-johnson syndrome
• pyoderma gangrenosum