Hereditary angioedema (HAE) is a nonhistamine-mediated process causing edema of the tissues of the upper airway, dermis, and subcutaneous tissue. As such it does not respond well to epinephrine, antihistamines, or glucocorticoids. Instead it is treated with attenuated androgenic hormones, episolone aminocaproic acid (EACA), or tranexamic acid, C1 esterase inhibitor, and fresh-frozen plasma. Medical or surgical management of the airway may be necessary in an acute situation. Minor trauma, such as that associated with dental procedures and psychologic stress, may precipitate an attack; however, swelling may not manifest itself for 12-48 hours after a procedure. Symptoms of angioedema may be exacerbated in these patients if they are given an angiotensin-converting enzyme inhibitor. This paper reviews this entity and presents 2 patients who presented for acute care with dental/oral surgical complaints.
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