నైరూప్య

Hereditary angioedema type II combined with other allergic pathology-case report

Svetlan Dermendzhiev, Tihomir Dermendzhiev, Angel Dzhambov

Hereditary Angioedema (HAE) is a rare disease, and its incidence varies across different populations. HAE belongs to a group of congenital immune deficiencies in the complement system. Deficiency of the C1-Esterase Inhibitor (C1-INH) is leading in the pathogenesis of type I HAE; in type II, which is less common, the level of C1-INH is normal or increased, but an incomplete inhibitor is produced. Various provoking factors can trigger a cascade of reactions leading to activation of the kinin system. Consequently, the clinical picture of angioedema unfolds. There is a wide range of swelling-inducing factors-e.g., mechanical, pharmacological, physical, occupational, toxic. However, according to the literature, there is insufficient clarity about the combination of HAE type II with other allergic diseases, specifically with bronchial asthma. In the current study, we present a clinical case that is expected to enrich the knowledge base and contribute to the specification of the diagnostic/therapeutic algorithm in such patients with combined immuno-allergic pathology.