Dr. Bhupin Singh Bakshi

Hereditary Angioedema is a rare skin related disorder and as the name suggests it is also a genetically inherited condition. This condition mainly afflicts the throat, abdomen and the limbs of the patient, with a certain kind of swelling. This swelling can become fatal if not treated on time. Here is everything you need to know about the condition and its treatment.

Causes: A rare genetic mutation of the SERPING1 gene can cause this condition. Usually, this gene is known to produce C1 esterase inhibitor, which is a kind of blood protein. This blood protein is an important element of one’s immune system and is usually involved in the biochemical reactions that control the body’s response to inflammation causing elements. This is done when the blood protein produces a hormone known as Bradykinin. If this blood protein does not function in a normal manner, then there is excessive production of this hormone, which leads to swelling and inflammation.

Type One: There are various types of Hereditary Angioedema that can affect various parts of the body. Type one is one of the most common kinds and can be found on the face, hands, abdomen, throat, as well as feet and genitals of the patient. The severity and frequency of attacks cannot really be predicted and the patient may suffer from attacks throughout his or her life. The best way to treat this type of Hereditary Angioedema is with the help of antihistamine drugs and prednisone which can fight allergic attacks.

Type Two: This kind of Hereditary Angioedema has the same symptoms as type one and is caused by a malfunction of the C1 inhibitor, rather than the Bradykinin hormone as one can see in type one. This condition can also be treated with anti-allergy drugs.

Type Three: This is very rare kind of Hereditary Angioedema. In fact, medical science is yet to find a conclusive body of knowledge that can point to the exact causes and symptoms of this condition. Coagulation is one of the main causes recognised so far. This condition is much more common in women than it is in men, and it usually shows up as facial swelling.

Timely Treatment: While there is no cure for this condition, one can prevent a flare up of the symptoms by taking C1 inhibitors and Bradykinin receptors as well as enzyme inhibitors. It is important to get early treatment for the symptoms and to have constant access to a hospital and its emergency unit. One should also keep track of attacks and symptoms and get regular check-ups done by a doctor to detect the earliest signs of the same to avoid fatalities.