Shirley W. was a patient I had often seen for minor complaints and skin checks. But after a call for an urgent appointment, she was now sitting on my exam table with a problem she had never had before. 
“Dr. Norman,” she asked worriedly, “What the heck is going on with me?”

It was obvious why she was so concerned. Shirley’s upper lip was grossly swollen, puffed up to almost three times the size of her lower lip. My 38-year-old patient otherwise looked healthy, with clear blue eyes and light blond hair pulled back into a designer clip. She was a sales and marketing manager, and though she’d had the swelling for only two days, she was afraid her distorted lip would make it hard to meet with clients.

I could sympathize. The swelling was disturbing even to me. In my career as a dermatologist, I’d had only a handful of patients with lips swollen to this size.

As I looked at her, the common diagnoses for a swollen lip flashed through my mind: Hives, infected salivary glands, food allergies, trauma, and irritation from certain cosmetics or chemicals can all cause severe swelling of the lips.

Shirley told me she hadn’t banged into anything or applied anything new to her lips, so that ruled out trauma and chemical irritation. She also said she had no history of food allergies.

In examining her I found that her tongue and gums were normal, which seemed to rule out an infection of the salivary glands, since it typically involves swelling throughout the mouth. I also couldn’t detect any facial paralysis that would indicate Bell’s palsy, an inflammation of the facial nerve that sometimes first presents as a swollen lip. “Have you had any other symptoms?” I asked, hunting for more clues.

“A few times over the years, parts of me have swelled up, like my hands,” she said. “But I just thought it was hives. My belly hurts. But that has been going on for years. I went to a gi specialist and he didn’t find anything wrong. But this lip swelling is new.”

I could not immediately see a link between the belly pain and her swollen lip. Often patients provide a cascade of extraneous information, hoping that a doctor will make a connection between unrelated facts. So 
I ignored the belly pain and focused on the possibility that Shirley might be suffering from a severe case of hives, or urticaria, which affects up 
to 25 percent of the population.

A Routine Diagnosis?


Hives are raised bumps on the skin that can be caused by allergic reactions to a number of triggers, including drugs, foods, insect stings, and infections. The reactions lead to the release into the bloodstream of chemicals known as histamines, which in turn produce skin swelling. The bumps can be single welts or can combine to form larger patches of raised skin. A related condition, angioedema, involves swelling in deeper layers of the skin.

The most common complaint of people with hives is severe itching that can make work difficult and cause sleeping problems. More seriously, urticaria in the airways can complicate breathing. Although most cases go away within several hours, the problem can recur off and on for as long as six weeks.

Some patients suffer from chronic and repeated episodes of urticaria, which account for approximately 
30 percent of hives cases. Although it can be difficult to tell the difference between an occasional and a chronic attack of hives, chronic hives’ lesions usually last longer, and recurrences can go on for more than six weeks. People suffering from chronic urticaria often experience an increase in thyroid autoantibodies, a condition in which the thyroid cells are attacked by the body’s own immune system, resulting in damage to the thyroid gland and persistent inflammation.

Since Shirley had complained of previous swelling in her hands, and since her lip had been swollen for two days already, I had a hunch she was suffering from chronic urticaria. I gave her a prescription for a strong antihistamine, which suppresses histamines and reduces swelling, and scheduled a follow up appointment for the next week.

But when Shirley came back to my office, her lip was still swollen. Puzzled that the antihistamine hadn’t taken care of it, I ordered a series of tests. Over the next week the results of her laboratory evaluation, including a routine blood chemistry profile and thyroid tests to check for autoantibodies, came back normal. I also sent her to an allergist for patch tests, in which the doctor places rows of various chemical patches on the back to determine if any specific substance elicits allergic inflammation of the skin. The results were all negative.

Adding to the diagnostic confusion was the mercurial nature of Shirley’s disease. When I saw her again about three weeks after the problem first started, her lip had almost returned to normal, but now her left eye was grossly swollen. “My eye is itchy and it keeps watering,” she told me. “I can’t do my job properly, and I really need to know what’s going on with me.” She also told me that her belly was still troubling her.