Intestinal Angioedema: Case Report and Literature Review

Abstract

Zemia Maria Camara Costa Ferreira, Anna Gabriela dos Santos Silva, Fausto Daniel Mendes Morel, Mayane Abrantes Veras de Freitas, Irami Araujo-Neto, Fabiana Lima Bezerra, Silvio Jose de Lucena Dantas, Amalia Cinthia Meneses Rego, Irami Araujo-Filho

Hereditary angioedema (HAE) is the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). However, it may also occur due to either increased activity of factor XII / estrogen levels or through an unidentified cause. It manifests the attacks of swelling involving the skin and / or the mucosa / sub mucosa of different organs. The attacks may be the result of a specific trigger or occur spontaneously. The intestinal angioedema is clinically presented with moderate or severe abdominal pain, associated with nausea, vomiting, diarrhea and / or ascites, and interpreted as an “acute abdomen”. The treatment is into three distinct phases: treatment for acute attacks, short-term prevention, and long-term prophylaxis. The 26-year-old woman with food and medication allergy presents with thirteen-year history of recurrent abdominal pain diffuse and associated with diarrhea, nausea and hands, lips and eyelids swelling. During this period, she did several exams and six laparoscopies that only revealed a small amount of free intraperitoneal fluid. Biochemical testing performed at that time revealed the C1 esterase inhibitor, decreased protein level, and C4 level, and she was, then, diagnosed in adults with intestinal involvement with HAE. After the adequate treatment and prophylaxis, she evolved with reduction of the number of attacks. The late diagnosis is associated with high morbidity. Therefore, it is extremely important the recognition and investigation of HAE with involvement in intestinal patients with recurrent attacks of unexplained abdominal pain.

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