Delayed diagnosis of hereditary angioedema

Hereditary angioedema (HAE) is a rare and potentially fatal disease that is important to recognize early. It is usually associated with low levels or impaired function of C1 inhibitor, which is involved in several inflammatory pathways. The treatment of HAE is very different from other causes of angioedema, emphasizing the importance of early and accurate diagnosis. The authors report the case of a patient who had symptoms starting in his teens but was not diagnosed until the age of 57 years. They also review the consequences of delayed diagnosis of HAE.