Angioedema in the Pediatric Population: A Practical Approach

Angioedema is characterized by subcutaneous and submucosal accumulation of fluids caused by increased permeability of vascular endothelium. In most cases, especially in pediatric age, the swelling is caused by the release of histamine and other vasoactive molecules by mast cells, both when it is associated with urticaria and when isolated. However, there are also forms mediated by bradykinin, including hereditary angioedema (HAE) due to C1-INH deficiency. This is a rare genetic disease that typically begins in childhood and is not always promptly identified. New variants of HAE with normal levels of C1INH have been discovered in recent years, whilst other mutations still remain unknown. This chapter aims to provide a practical approach to the differential diagnosis of angioedema without wheals, with particular regard to the pediatric population and to the main therapeutic indications in these forms.