Angioedema Presenting as Infant-Onset Primary Lymphedema.

Primary lymphedema typically manifests as extremity swelling that is chronic, progressive, and incurable. We present a patient with four-limb and facial swelling who was referred for a possible diagnosis of primary lymphedema. The patient eventually was diagnosed with idiopathic spontaneous angioedema. This case highlights distinguishing characteristics between the two disease processes and the importance of recognizing idiopathic spontaneous angioedema on the differential for primary lymphedema.