Zoe was a 32-year-old woman, with a normal BMI, she did not smoke, ate well and took plenty of exercise. She was not atopic and did not have drug allergies or any significant family history. She suffered from tonsillitis as a child but it had not warranted a tonsillectomy.
She came to the surgery two years ago with shingles of the face. This settled but left her with intermittent symptoms of trigeminal neuralgia.
A few months later she had a sore throat and felt unwell. She developed puffy eyes, swelling of her nose, cheeks and lips to the point that her normal facial features were excluded.
There was no swelling of the tongue and no respiratory distress. She was treated at A&E with antihistamines, which settled her symptoms.
Further questioning revealed that Zoe frequently suffered from throat infections and gastroenteritis, which seemed to trigger trigeminal symptoms. She always made a full recovery, usually without antibiotics. She was also now on oral loratadine.
When followed up at the surgery Zoe had some evidence of dermatographism on her skin. Her throat examination and her lymph nodes were normal. Blood tests, including liver, kidney, glucose and thyroid tests, were normal. She had normal immunoglobulin levels and white cell count (though with increased lymphocytes).
I referred her to ENT to exclude a tonsillar cause for her head and neck infections. She underwent a nasal endoscopy and pharyngolaryngoscopy. This was normal except for some nasal secretions.
The specialist attributed her symptoms to polyneuropathy with involvement of the glossopharyngeal nerve following herpes zoster infection.
Zoe is now taking amitriptyline for her facial symptoms as well as antihistamines.
Urticaria and angioedema
Angioedema is defined as deep submucosal swelling from increased vascular permeability. Most forms of angioedema are thought to be idiopathic.
Angioedema can occur with or without urticaria and can affect the skin, mouth, larynx and GI tract. Both are similar in their pathogenesis, although angioedema is deeper within the tissues.
Urticaria is often spontaneous or induced by minor trauma or temperature changes. This is mast-cell mediated with histamine released into the blood causing increased cell permeability. Food allergies can be excluded on the basis of a history without the need for investigations.
Angioedema without urticaria is either hereditary (C1 esterase deficiency) or acquired.
C1 esterase deficiency causes formation of substances (such as bradykinin) that promote vasodilation. Acquired angioedema usually occurs later on in life and is associated with autoimmune or lymphoproliferative disorders. ACE inhibitors can cause drug-induced angioedema by increasing bradykinin levels in the blood.
Management of angioedema with or without urticaria is usually with antihistamines such as cetirizine or loratadine, the dose of which can be doubled if necessary. Ranitidine (150mg twice daily) can also be added to give both H1 and H2 histamine receptor blockade.
All patients on ACE inhibitors should be changed to angiotensin II antagonists.
In C1 esterase deficiency there may be a clear history of hereditary angioedema. If this is suspected, patients should be referred to an allergy clinic for a C4 complement levels test.