It’s a long weekend, and you’re on a small vacation with your family. The next morning you wake up earlier than usual, excited, and you go to wake up your child. You turn on the lights and immediately notice your little one’s eyes and lips are puffed up.  Her face is swollen. It’s as if someone had pumped air into face her overnight. It’s terrifying. Your child is having an attack of angioedema. Call 911.

ANGIOEDEMA: THE WHAT & WHY

Angioedema is an acute medical condition characterized by sudden and rapid swelling of deeper skin layers. Generally, this is seen around the eyes and lips. When it happens in children, the condition can be scary, as the swelling may even prevent the child from opening his or her eyes.

Angioedema can be either congenital or acquired, depending on the cause. Hereditary angioedema (HAE) is a genetic condition inherited from parents or grandparents. The symptoms may manifest  early childhood. [1] The exact cause of hereditary angioedema is not known, but even mild trauma like dental work, surgery, or other stimuli can initiate an episode. [2] On the other hand, acquired version of this condition can be an autoimmune response or without any identifiable cause. This is called “idiopathic”. [3]

SYMPTOMS

HAE generally affects the limbs, the trunk, the throat, and the genitals. The swelling is generally brawny with no associated urticaria (hives), itching, or pain. Only about 25% of patients have a positive family history. It can also manifest as an attack of severe abdominal pain along with nausea, vomiting, and occasionally, watery diarrhea. Patients suffering from HAE may have one episode per month on average, but there are patients who have weekly episodes. Conversely, some children may have just one or two episodes per year.

Acquired angioedema can affect any part of the body, but the abdominal symptoms are generally not as severe. Acquired angioedema can be associated with hives, and the swelling can be either painful or itchy.

DIAGNOSIS & MANAGEMENT

Crucially, angioedema is a medical emergency, as the swelling can lead to airway obstruction and suffocation. Your child must be rushed to the emergency department at once.

After stabilizing the child with antihistamine injections, blood tests for IgE levels and a skin-prick test might be performed to find the exact allergen leading to the hyperactive immune response. Depending on the cause, you and the child will be educated about sources of exposure to the allergen and how they can be studiously avoided. Finally, you’ll learn what precautions to take to prevent or manage such an episode in the future.

Image Courtesy James Heilman, MD/ CC 3.0

References:

  1. Farkas H. “Pediatric hereditary angioedema due to C1-inhibitor deficiency”. Allergy, Asthma Clinical Immunology. 2010 Jul 28. 6(1):18. [Medline].
  2. Bork K, Barnstedt Se (August 2003). “Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema”. Journal of the American Dental Assoc.134 (8): 1088–94. doi:10.14219/jada.archive.2003.0323. PMID 12956349
  3. Axelrod, S; Davis-Lorton, M (2011). “Urticaria and angioedema”. The Mount Sinai journal of medicine, New York 78 (5): 784–802. doi:10.1002/msj.20288.PMID 21913206.

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