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.