Abstract
Angioedema is caused by a local increase in vascular permeability induced by bradykinin or mast cell mediators. The main challenge of the differential diagnosis of angioedema lies in the fact that different pathophysiological processes may lead to a very similar clinical picture. Moreover, verification of the type of angioedema is crucial for selection of appropriate therapy. In 2020, a school for doctors Chronic urticaria: scientific and medical accomplishments and practical aspects of patient management was held on the basis of the National Research Center Institute of Immunology of the FMBA of Russia as part of the work of the reference center that provides expert assistance to patients with urticaria (GALEN UCARE). A clinical case series of related patients with hereditary angioedema due to a mutation in the PLG gene was presented to demonstrate the challenges of the differential diagnosis between types of angioedema. This article focused on the description of this series.