Background
{mosimage}On physical examination, the patient's vital signs are a temperature of 98.9°F (37.2°C), blood pressure of 140/67 mm Hg, heart rate of 77 bpm, respiratory rate of 18 breaths/min, and an O2 saturation of 99% while he is breathing room air. The patient appears well and is in no acute distress. The head, neck, and throat examination reveals marked swelling of the right side of the tongue and the floor of his mouth (see Image). 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 the heart sounds are regular and without murmurs. The abdomen is soft and nontender. The extremities show no signs of cyanosis or edema. A detailed skin examination fails to demonstrate any abnormal lesions; in particular, no urticarial lesions are identified.
What is the diagnosis?
Hint
This is an uncommon presentation of a relatively common bilateral phenomenon.
Authors:
Gil Z. Shlamovitz, MD, Attending Physician, Emergency Department, Windham Community Memorial Hospital, Willimatic, CT. Assistant Clinical Professor, Department of Traumatology & Emergency Medicine, University of Connecticut.
eMedicine Editors:
Rick G. Kulkarni, MD, FACEP, Assistant Professor, Yale School of Medicine, Section of Emergency Medicine, Department of Surgery, Attending Physician, Medical Director, Department of Emergency Services, Yale-New Haven Hospital, CT
Answer
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. Urticaria and angioedema 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; the pathology 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:
1. 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)
2. Bradykinin-mediated group, which may involve hereditary or acquired C1 esterase inhibitor (C1INH) deficiency or malfunction or may involve angiotensin-converting enzyme (ACE) inhibitors
3. Complement-mediated group, in which patients may have serum sickness, necrotizing vasculitis, or a reaction to a transfusion of blood products
4. Nonimmunologic group, in which the 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)
5. Idiopathic
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 (an anabolic steroid) and danazol (a 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 this case was treated with intravenous H1 and H2 blockers and glucocorticoids. He was observed in the ED for 4 hours, and his tongue swelling markedly improved. At discharge, a 5-day course of antihistamines, prednisone, and self-injectable epinephrine (Epi-Pen) was prescribed. C1INH levels and function studies were ordered from the ED, and the results were reported to be within normal limits. The patient was referred to an immunologist.
References:
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3. Kasper DL, Braunwald E, Fauci A, et al. Harrison's Principles of Internal Medicine. 16th ed. New York: McGraw-Hill Professional; 2004.
4. Lehmke J. Angioneurotic edema with unilateral tongue swelling in enalapril therapy. Med Klin (Munich) 1994 Sep 15;89(9):508-10. [MEDLINE: 7968889]
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