A 45-year-old man presents to the emergency department with swelling of the tongue that started about 30 minutes before his arrival. The patient denies having shortness of breath, a sore throat, drooling, or globus sensation. He also denies ingestion of new foods, pills, or supplements and any exposure to new items such as soap, tooth paste, deodorants, and laundry detergents. The patient is not taking any medications and is allergic to penicillin. He reports a history of urticaria and 1 episode of an allergic facial swelling that was successfully treated with antihistamines and steroids.
On physical examination, the patient's vital signs are a temperature of 37.2°C, blood pressure of 140/67, heart rate of 77 beats per minute, respiratory rate of 18 breaths per minute, and an O2 saturation of 99% while he is breathing room air. The patient appears well and is in no acute distress. Head, eyes, ears, nose, and throat examination reveals marked swelling of the right side of the tongue and the floor of his mouth (see Image). Of note, the patient has no difficulty opening his mouth, and the floor of his mouth is soft to palpation. The uvula is visualized by using a tongue depressor; it is midline and not swollen.
The lungs are clear to auscultation without any wheezing or rales, and hearts sounds are regular and without murmurs. The abdomen is soft and not tender. The extremities show no signs of cyanosis or edema. Detailed skin examination fails to demonstrate any abnormal lesions; in particular, no urticarial lesions are identified.
What is the diagnosis?
Idiopathic unilateral angioedema of the tongue: The image shows angioedema limited to the right side of the tongue. Urticaria and angioedema may appear separately or together as cutaneous manifestations of localized nonpitting edema. The process may also occur on mucosal surfaces of the upper respiratory or GI tract.
Angioedema is a well-demarcated, localized edema involving the deep layers of the skin, including the subcutaneous tissue. The pathology of urticaria is usually characterized by edema of the dermis in urticaria; that of angioedema is characterized by edema of the subcutaneous tissue and dermis. In affected areas, collagen bundles are widely separated, and the venules are sometimes dilated. The perivenular infiltrate may consist of lymphocytes, eosinophils, and neutrophils that are present in various combinations and numbers throughout the dermis.
The 5 major etiologic groups for urticaria with or without angioedema are the following:
Immunoglobulin E (IgE)–dependent group, which may involve specific antigens (eg, foods, drugs, pollens, venoms), physical stimulation (eg, cold, vibration, exercise), or certain autoimmune disorders (eg, lupus erythematosus, cryoglobulinemia, juvenile rheumatoid arthritis, autoimmune thyroid disease)
Bradykinin-mediated group, which may involve hereditary or acquired C1 esterase inhibitor (C1INH) deficiency or malfunction or angiotensin-converting enzyme (ACE) inhibitors
Complement-mediated group, where patients may have serum sickness, necrotizing vasculitis, or a reaction to a transfusion of blood products
Nonimmunologic group, in which disease is related to direct mast-cell activators (eg, opiates, antibiotics, radiologic contrast material) or agents that alter arachidonic acid metabolism (eg, nonsteroidal anti-inflammatory drugs, azo dyes, benzoates)
The initial goal of therapy is airway management and attention to the patient's ventilation and oxygenation. The most skilled person available must handle airway interventions if they are necessary because oral obstruction is often massive. The treating physician should be ready to perform a surgical airway intervention when attempts to secure an oropharyngeal and/or nasopharyngeal airway fail.
After the patient's airway is secured, pharmacotherapy for nonhereditary angioedema usually involves a short course of H1 and H2 blockers as well as steroids. In advanced or progressive cases with airway compromise or in cases that are progressing despite adequate conservative pharmacotherapy, subcutaneous epinephrine can be given. Hereditary angioedema is more refractory to subcutaneous epinephrine, antihistamines, and steroids than nonhereditary angioedema. Stanozolol (anabolic steroid) and danazol (gonadotropin inhibitor) may be used in the acute phase of an episode of hereditary angioedema. C1INH should be replaced during moderate-to-severe episodes of hereditary angioedema; it is transfused as a C1INH concentrate or as fresh-frozen plasma.
The patient in our case was treated with intravenous H1 and H2 blockers as well as glucocorticoids. He was observed in the emergency department for 4 hours, when and his tongue swelling markedly improved. At discharge, a 5-day course of antihistamines and prednisone and self-injectable epinephrine (Epi-Pen) were prescribed. C1INH levels and function studies were ordered from the emergency department, and the results were reported to be within normal limits. The patient was referred to an immunologist.