Hereditary angioedema associated with familial mediterranean fever in a Jordanian family

Abstract EN

Hereditary angioedema is an autosomal dominant disease due to a deficiency of antigenic or functional CI esterase inhibitor.

In this report, three family members ; a female aged 36 years and her two children ; a six-year-old son, and a five-year-old daughter, in addition to her brother had recurrent attacks of angioedema affecting the face, the larynx and the abdomen in the absence ofpruritis.

All of them had episodes of fever, arthritis, pleuritis or severe abdominal pain and were diagnosed to have familial mediterranean fever based upon the clinical picture and the response to variable doses of colchicine.

It was noticed that the precipitating factors for an attack of angioedema were anxiety, stress and interestingly any attack of familial mediterranean fever that could be a stressful medical condition.

The combination of hereditary angioedema and familial mediterranean fever was not reported internationally using a Medline search since 1985.