Acquired angioedema (AAE) is a rare disorder that has been categorized
into two forms, AAE-I and AAE-II. AAE-I is associated with other dise
ases, most commonly B-cell lymphoproliferative disorders. AAE-II is de
fined by the presence of an autoantibody directed against the C1-inhib
itor molecule. Differentiating AAE-I from AAE-II is vital because diff
erent therapeutic interventions are required for each type. This revie
w summarizes the clinical aspects, pathophysiology, and management of
AAE compared with the types of hereditary angioedema.