Angioedema as a systemic disease

Angioedema is a clinical entity defined as self-limiting edema localized in the deeper layers of the skin and mucosa and lasting for several days. Angioedema can be provoked by bradykinin and/or mast cell mediators, including histamine. Four types of acquired and three types of hereditary angioedema have been identified. The most obvious form of angioedema associated with other systemic disease is acquired angioedema due to C1-inhibitor deficiency. It is characterized by acquired consumption of C1 inhibitor and various underlying disorders, such as multiple myeloma, chronic lymphocytic leukemia, rectal carcinoma, and non-Hodgkin lymphoma. Suspected cases need an accurate differential diagnosis to exclude all other types of acquired and hereditary angioedema.