In recent years there has been considerable research done on a clinical syndrome termed hereditary angioedema. This syndrome is caused by low or nonfunctional levels of C1-INH (C-1 esterase inhibitor), an important enzyme in the body. The syndrome is characterized by episodes of areas of swelling and discoloration of the skin that do NOT itch, abdominal pain and/or vomiting, swelling of the tissues of the lips, tongue, throat or voicebox, and usually with a history of another family member with the disease. 

Certain blood tests can now accurately determine which one of the three known types of this syndrome is present. This is important for effective treatment as specific medicines are given based on which type of hereditary angioedema is diagnosed. In the past, various medicines such as antihistamines, steroids, and male hormones have been used, without great success and with considerable side effects, to treat this disorder. Recently three medications have been developed that more effectively treat this potentially dangerous disease.  However, one can only be given intravenously, and the other two are given by subcutaneous injection. These medicines all have fewer side effects and are now available for self administration by the patient, after proper instruction by their allergist or doctor.

The importance of recognizing the difference between this syndrome and a conventional contact or food allergy reaction is important to allow rapid and effective treatment when an outbreak occurs. If you or a family member has episodes of abdominal pain, nausea, or vomiting with recurrent swelling of the skin of the extremities, face, or mouth that does NOT itch please call our office for an evaluation and lab testing to rule out hereditary angioedema. More information about the causes, diagnosis, and treatment of this disease is presented in the references listed below.  

 

http://www.haea.org/patients/treatments  

http://www.allabouthae.com/consumer/

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Suite #200
Hoover, AL 35244