Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review*

Anais Brasileiros de Dermatologia

Amanda Rodrigues Miranda¹  Ana Paula Fusel De Ue¹  Dominique Vilarinho Sabbag¹  Wellington De Jesus Furlani¹  Patrícia Karla De Souza¹  Osmar Rotta¹ 

关键词: Angioedemas;    hereditary;    Estrogens;    Hereditary angioedema type III;    Therapeutics;    Angioedemas hereditários;    Angioedema hereditário tipo III;    Estrogênios;    Terapêutica;   

DOI  :  10.1590/abd1806-4841.20131818

来源: SciELO

【 摘 要 】

In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

【 授权许可】

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