Isolated uvular angioedema: Quincke’s disease

A 39-year-old male patient was admitted to the emergency department with the complaints of difficulty swallowing, a sensation of something “stuck” in his throat and a swollen uvula that he had seen in the mirror while brushing his teeth. His complaints had started 1 day before with sore throat as well as difficulty and pain when eating and drinking. He denied any fever, cough or breathing difficulties. His vital signs were stabile with blood pressure 120/70 mmHg, heart rate 86/min, respiration rate 12/min and body temperature 36.6°C. On physical examination, his lung sounds were normal. No lymphadenopathy was noted, but his uvula was erythematous and edematous (Fig. 1). No tonsillar hypertrophy was seen. His laboratory tests showed no significant results; there was no leukocytosis or any alterations in the neutrophil, basophil or eosinophil counts. The postero-anterior chest x-ray was normal. A bedside nasopharyngeal scope revealed a normal epiglottis and vocal cords, with significant uvular edema. He had no medical history of trauma, known food or drug allergy, asthma or frequently repeating infections. The only remarkable point in his medical history was that 1 week before he had been prescribed oral pinaverium bromide 50 mg (Dicetel®) for irritable bowel syndrome. He was treated with intravenous pheniramine hydrogen maleate (Avil®) and dexamethasone (Dekort®) in the emergency department and prescribed oral antihistaminic tablets for the next 48 h.

Fig. 1

Oropharyngeal examination of a 39-year-old patient: isolated uvular angioedema (the patient's consent was obtained for the photograph)

Isolated uvular angioedema was first defined by Quincke in 1882 [1]. Isolated uvular angioedema, or Quincke's disease, is a relatively rare presentation of angioedema of the upper airway [2]. Several causes of uvular edema have been described, including hereditary angioedema, trauma, inhalation exposure, medication reactions and infectious causes [3, 4]. Isolated uvular angioedema is usually caused by a type I hypersensitivity reaction [2, 3]. This should be differentiated from uvulitis, which is infectious and frequently has concomitant epiglottitis. Direct visualization or a lateral neck plain radiograph should be considered to help rule out epiglottitis [3, 5]. In this situation, the primary strategy should invovle maintaining the airway. In spite of being a rare condition, uvular edema may cause obstructive respiratory distress and require immediate airway care. The general treatment strategies in the emergency department consist of intravenous H1 and H2 histamine blockers, corticosteroids and infrequently epinephrine [2]. Dexamethasone has been considered the medication of choice considering its potent anti-inflammatory properties and long half-life.