Pam Pezzin was walking in the woods on a winter day back around 1989 when she broke out in hives all over her body, including her stomach, arms, legs and face.
Over the years, she continued to break out in bumps when it was cold outside, and she suffered from symptoms of asthma, too.
She had a period of remission, but the reactions resumed about 10 years ago. "It got severe again," she said.
None of the doctors could figure out exactly what was wrong with her until she saw allergy, asthma and immunology expert Deborah Gentile in 2009.
Dr. Gentile, director of research, Division of Allergy, Asthma and Immunology at Allegheny General Hospital in Pittsburgh, told Pezzin, now 40, she has cold-induced urticaria, urticaria being medical-speak for hives. Cells in her skin were releasing chemicals that cause itching, swelling and redness.
Her allergic response to cold is so severe that sometimes her lips swell, her airways constrict and she has trouble breathing. "I've been to the emergency room on multiple occasions," said Pezzin, a social worker. "They did administer epinephrine and breathing treatments, always breathing treatments, because I'm virtually closing up."
Cold-induced urticaria is one of several categories of allergic reactions called physical urticarias.
"It's an interesting phenomenon. There's a whole group of environmental factors that can cause urticaria," Gentile said.
"It's a medical condition in which the patient breaks out in hives, which are induced usually by various physical or environmental stimuli such as heat, cold, pressure applied to the skin, water, vibration, sunlight, exercise," said Dr. Andrej Petrov, medical director of allergy and clinical immunology in a division of pulmonary allergy and critical-care medicine at the University of Pittsburgh Medical Center. "These are all various, but usually there's a trigger that is usually readily identifiable."
For example, Janet Zahorsky, another one of Gentile's patients, has cholinergic, or heat-induced, urticaria. When she is exposed to heat, the 22-year-old College of Wooster junior gets hives, and the reaction is exacerbated by food allergies. If she is exposed to certain foods and goes out in the heat, her hives are worse than if they were just heat-induced.
In some patients who get hives, it is difficult for the allergist/immunologist to identify the trigger. "But with physical urticaria most of the time you can sort of identify the trigger by doing a history from a patient," Petrov said. "Usually patients will let you know. ... They'll say, 'In cold weather I break out in hives. When I'm running and sweating I break out in hives. I carry a bag and where I put pressure I break out in hives,' so usually patients will have a clue."
If taking a history doesn't work, sometimes the doctors can provoke the allergic response. For example, to test for cold urticaria, they'll put an ice cube in a plastic bag and press it to bare skin for five minutes. "Ten minutes later, they'll break out in a huge hive," Petrov said.
The trigger for this kind of allergy causes mast cells, or cells in connective tissue, to burst and release irritating chemicals like histamine into the affected area, causing itching, swelling and fluid leaking from cells.
Cold urticaria is divided into three subtypes and can be dangerous.
"Most common is primary, or essential, cold urticaria, and the hive is usually localized to the area of exposure," said Allegheny General Hospital dermatologist Diane Inserra. Patients usually get it in early childhood and can have it for many years. "It can be very severe, and if somebody were just to jump into ice-cold water and weren't treated, (they can) have cardiovascular collapse and die."
Treatment is usually a combination of antihistamines, although occasionally, Dr. Inserra said, doctors have tried using desensitization, slowly exposing the patient to cold over increasing parts of the body.
In secondary cold urticaria, there usually is an underlying disease, like multiple myeloma, hepatitis or mononucleosis. Patients "don't just get hives but systemic reactions like headaches and very low blood pressure," she said, "and they can pass out when they're exposed." Antihistamines generally don't help much; instead, the allergist works with the patient's personal-care physician to diagnose and treat the underlying disease.
The third subtype of cold urticaria is familial or inherited urticaria. "In this the hives definitely burn more than itch," Inserra said. They also last for about 48 hours, as compared to the 24 usual hours of regular hives. The treatment is anabolic steroids because antihistamines "don't work at all."
There are subtypes for some of the other physical urticaria, too, but "they're a little less complicated than cold urticarias," Inserra said. The exception is heat urticaria, which, she said, can be very complicated to treat.
In most cases of cold urticaria, over-the-counter antihistamines do the trick. "Most do very well.
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