Edema angioneurótico hereditario / Hereditary angioneurotic edema

RESUMO

Angiodema, or angioneurotic edema (AE), is a condition characterized by the extravasation of fluid and its entry into interstitial tissues that may occur on any level. It becomes a vital urgency when it compromises the air way. Its pathophysiology is complex, with two recognizable pathways. One is mediated by mast cells and the other is not. The hereditary angioneurotic edema(HAE) belongs to this last group. A patientwith diagnosed HAE with air way involvement, that consults again within a year because of a compartimental sindrome in the upper limb, is presented in this article. Aspects about epidemiology, pathophysiology, clinical manifestations and laboratory in HAE, as wel as current available acute and chronic treatment alternatives are discussed