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 4-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 2 disease processes and the importance of recognizing idiopathic spontaneous angioedema on the differential for primary lymphedema.