Hereditary angioedema (HAE), characterized by recurrent episodes of painful and/or disabling angioedema, is commonly mistaken for other conditions such as allergic (mast cell-mediated) angioedema, but has very different treatment requirements.

Although rare, dermatologists are likely to encounter patients with HAE at some point over the course of their clinical practice due to the fact that HAE episodes typically involve subcutaneous swelling and sometimes erythema marginatum.

The key distinguishing diagnostic aspects of HAE-related angioedema include an absence of wheals/urticaria; unresponsiveness to typical interventions (e.g., epinephrine, high-dose antihistamines, corticosteroids), and laboratory abnormalities in C1 inhibitor quantity and/or function and C4 level (depending on the subtype of HAE).

Multiple disease-specific treatments are available for all HAE management strategies, which may include acute treatment of attacks, short-term prophylaxis prior to potentially triggering events, and routine or long-term prophylaxis to prevent attacks on an ongoing basis.