Clinical and biological response to rituximab treatment in 3 patients with acquired C1-inhibitor deficiency

Acquired C1 inhibitor (C1-INH) deficiency (ACID) is a rare cause of recurrent subcutaneous and submucosal edema without urticaria. The mechanisms of ACID include excessive C1-INH consumption because of an underlying lymphoid malignant tumors (often undiagnosed) or the presence of anti–C1-INH autoantibodies, often with an isolated monoclonal gammopathy or a lymphoproliferative disorder.1–3 The primary therapy for ACID is treatment of the underlying condition; however, management is complex, and all patients must have a medication for acute angioedema.

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