Angioedema can occur in isolation, accompanied by urticaria, or as a feature of anaphylaxis in mast cell-mediated disorders, bradykinin-mediated disorders, as well as in others with unknown mechanisms, such as infections, rare disorders, or idiopathic angioedema. In mast cell-mediated angioedema, other signs and symptoms of mast cell-mediator release are frequently seen. However, clear evidence of mast cell degranulation may be absent in histaminergic angioedema. Bradykinin-induced angioedema is not associated with urticaria or other symptoms of type I hypersensitivity reactions. For many of the known triggers of angioedema, the mechanism is unclear. While mast cell and bradykinin-mediated angioedema are relatively well defined in terms of diagnostic and therapeutic approach, angioedema with unknown mechanisms represents a challenge for patients and clinicians alike. Elucidating the clinical pattern and the possible causes of isolated angioedema is the key to a correct diagnosis. This review summarizes the causes, and clinical features of angioedema, with a focus on isolated angioedema.
Keywords: acquired angioedema; bradykinin-mediated angioedema; hereditary angioedema; histaminergic angioedema; idiopathic angioedema.