A 45 year-old Caucasian male presents with approximately 12 episodes of tongue swelling over the last year. Each episode occurs upon awakening in the morning, is preceded by tongue tingling, and is not associated with swelling in other locations of his body. He has no associated airway obstruction but does have difficulty talking and closing his mouth due to the degree of swelling. He has no history of urticaria and does not develop hives in conjunction with tongue swelling. He denies any other signs or symptoms of anaphylaxis.
The patient has been evaluated in the emergency room for many of these episodes and treated with diphenhydramine, prednisone, and ranitidine with improvement in symptoms within an hour of receiving medication. He has also self-treated many episodes with diphenhydramine at home with quick resolution of symptoms. Other times, he has had symptoms of only tongue tingling and is able to abort an episode of swelling with quick treatment with diphenhydramine. He has no history of allergic rhinitis, allergic skin disorders, or food allergy. He has not noticed any specific seasonality, food ingestion, rhinitis, or ocular symptoms associated with episodes.
On review of systems, patient complained of frequent indigestion and heartburn for the last year but denied halitosis, acidic taste in his mouth, or cough. He has no history of mouth sores or ulcers, dental caries, or gingivitis. Laboratory evaluation included normal complete blood count, chemistries, and liver function tests. Thyrotropin was 2.7 mIU/ml (0.27-4.2), C4 25.3 mg/dL (10-40), C1 esterase inhibitor quantity 12 mg/dL (11-26), and C1 esterase inhibitor function >100% (>68%).
After initial evaluation, daily antihistamine therapy was initiated and patient had complete resolution of episodes of angioedema with the exception of one episode after missing two days of therapy. Helicobacter pylori IgG was also evaluated due to the patient's complaints of heartburn. With a positive history and an elevated H. pylori IgG at 1.36 u/mL (1-1.09), patient was treated with triple drug therapy including: amoxicillin, clarithromycin, and omeprazole. After therapy for H. pylori eradication, patient discontinued treatment of fexofenadine and within 1 day had an episode of angioedema of the tongue. He restarted daily fexofenadine and has remained free of episodes of angioedema.