Intravenous immunoglobulin treatment in laryngeal pemphigoid

We report a 29-year-old white woman who presented with oral involvement and subsequently developed severe and extensive laryngeal involvement with mucous membrane pemphigoid. She could not tolerate systemic corticosteroids due to side-effects. Dapsone improved only the oral disease, but the laryngeal disease remained active and progressive. She refused immunosuppressive agents, because she wanted to conceive. The acute severe widespread laryngeal pemphigoid was accompanied by severe relentless coughing and dysphonia. The patient was treated with intravenous immunoglobulin (IVIg) as monotherapy, which led to complete resolution of the symptoms and the laryngeal lesions, as seen on repeated direct laryngoscopies. The normal anatomy and function of the larynx was restored, and scarring did not occur. The patient did not develop any side-effects to the IVIg treatment, which led to disease remission. While on IVIg treatment, she delivered a healthy child. She remained in remission after 5 years of follow-up.