Facial Angioedema and Systemic Lupus Erythematosus: Case Report

Non-medication–related acquired deficiencies of C1 esterase inhibitor (C1-INH) can cause the facial acquired angioedema (AAE) seen in systemic lupus erythematosus (SLE). The defect can originate from a lymphoproliferative disease (LPD) that catabolizes C1-INH or from circulating antibodies that inactivate C1-INH. This report describes a third and rare variety of facial AAE originating in SLE in which there was no LPD or circulating antibodies to impede C1-INH activity.