A Case of Acquired C1 Esterase Inhibitor (C1-INH) Deficiency As the Presenting Manifestation of Common Variable Immune Deficiency (CVID)

Acquired angioedema is a rare syndrome of recurrent episodes of angioedema without urticaria caused by an acquired deficiency of C1-INH. CVID is a heterogeneous primary immunodeficiency that is characterized by susceptibility to infections and immune dysregulation including autoimmunity. Here we offer a rare case report of a patient with angioedema due to acquired C1-INH deficiency presenting as the initial manifestation of CVID.