Years of hives and fevers traced to a startling cause

There was little that Beth Sternlieb’s doctors in Los Angeles could say with certainty about her baffling illness, but one thing was clear: Although manageable for years, it had gotten significantly worse.

For nearly two decades, Sternlieb was plagued by flu-like episodes that began with headaches and abdominal pain accompanied by fatigue, muscle aches and diarrhea. Within a day, a red, blotchy rash covered his abdomen.

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A yoga and meditation teacher who worked in the pediatric pain program at the University of California, Los Angeles, Sternlieb had undergone numerous tests that failed to reveal the reason for this unidentified illness that occurred two or three times. per year and lasted approximately five days. In 2004, after 17 years, the illness began to manifest more frequently and Sternlieb never fully recovered between episodes. A year later, she developed a high fever, chills and exhaustion that lasted five months and left her bedridden.

The surprising and highly unusual cause was eventually identified after Sternlieb underwent an operation that ultimately cured her.

“It’s a good thing my abdomen turned red, because it got my doctors’ attention,” Sternlieb said recently. “Something was definitely wrong, but no one imagined it. »

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Bad case of flu

The first episode occurred in December 1987, two weeks after the birth of Sternlieb’s second child. “I became sicker than I had ever been,” said Sternlieb, then 37. “It was flu season and that year was a bad flu season,” so doctors attributed his illness to the flu.

Six months later, the disease returned, a trend that lasted for years.

At first, Sternlieb didn’t pay much attention to the small red dots that covered his abdomen. The rash looked like a sunburn but was neither itchy nor painful. Doctors ultimately decided it was hives, a common skin condition that can occur as an allergic reaction to foods or medications; often its cause is never discovered.

Her GP referred her to a rheumatologist, a doctor specializing in the treatment of autoimmune diseases, whom she consulted for several years. He ordered blood tests that he said suggested the presence of an unspecified autoimmune disease in which the body attacks itself by mistake.

Over the years, Sternlieb noticed that these episodes seemed to occur during periods of stress “both good and bad,” including travel, parties and lack of sleep. “I thought it must have a psychological component,” she said.

She learned to incorporate these struggles into her life, relieved that no one found anything serious. She hoped doctors would find out what was wrong so they could treat and eradicate the disease.

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Travel story

In 2005, Sternlieb’s serenity was shattered by a sharp deterioration in his health.

That summer, she fell seriously ill and did not recover. Her fever periodically rose to 104 degrees, and she suffered from profuse night sweats and profound weakness and fatigue. She lost 15 pounds and, unable to work, spent most of her time in bed or on the couch. The rash that was confined to his abdomen spread to his neck and torso. Blood tests showed high levels of inflammation and high white blood cell counts.

Sternlieb began to see a new generation of specialists. An infectious disease doctor combed through his travel history, which included a trip to India years earlier, ultimately ruling out malaria and other parasitic infections. Doctors considered and rejected various diagnoses, including fever of unknown origin, which can be associated with certain autoimmune diseases; familial Mediterranean fever, an inherited genetic disease that causes recurrent fevers and inflammation; as well as HIV and hepatitis.

This left infection or allergy as possible causes. The latter case seemed unlikely despite the recurring hives, said Raffi Tachdjian, then an allergy and immunology researcher at UCLA and one of the doctors Sternlieb consulted.

“Hives usually last 24 hours and don’t look like this, which was chronic,” he recalls. “We had to dig deeper into anything unusual. … It seemed like there was something heating up somewhere” that triggered a reaction from Sternlieb’s immune system.

“We see this in the sinuses where antibiotics don’t reach the infected tissue” and result in a latent infection that becomes virtually impossible to eradicate with drugs, he added.

A CT scan ordered by the infectious disease doctor showed multiple uterine fibroids, common benign tumors that require no treatment unless they cause problems. The scan showed that one of the fibroids had grown very large and was possibly degenerated (dying) or necrotic (dead), which happens when a tumor loses its blood supply.

A degenerative fibroid can grow very large very quickly. But doctors also worried about the possibility of a rare cancer, such as leiomyosarcoma, that develops in smooth muscle, particularly in uterine tissue. None of her doctors, including her new gynecologist Jessica Schneider, knew if her long-standing illness and the fibroids were related.

And what explains hives, which is not associated with fibroids or this cancer?

“It didn’t seem obvious that a fibroid could cause this,” said Schneider, a member of the Cedars-Sinai Medical Group. “But it didn’t look like a typical fibroid and I recommended removing it.” Sternlieb, who said he feared he would still be sick after a hysterectomy, agreed.

During the December 2005 operation, Schneider removed eight fibroids. The largest measured 11 centimeters, the size of a large grapefruit.

Nearly 20 years later, Schneider vividly remembers its unique characteristics. Typically, a fibroid is a solid lump of muscle, she said. It was full of pus which gushed out explosively when touched with a scalpel.

“It was crazy,” said Schneider, who had never seen anything like it before and hasn’t seen it since. She gave him antibiotics and took a culture which she sent to the pathology lab for analysis.

Tachdjian remembers Schneider calling him right after his surgery was over to tell him what she had found.

“I said to myself, ‘We need to know what grew it,’” Tachdjian said. “We were keeping our fingers crossed that the surgery would take care of it, no matter what. But only time will tell.

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‘A pretty nest’

A few weeks later, the first question was answered. The culture revealed an unknown strain of salmonella, a common bacterial infection usually caused by contaminated food. The Centers for Disease Control and Prevention estimates that it causes more than 1.3 million illnesses per year and results in more than 26,000 hospitalizations and 420 deaths. Neither Sternlieb nor her doctors knew how or when she contracted salmonella, known to cause hives if it takes hold in the intestine, Tachdjian said.

In Sternlieb’s case, the bacteria had burrowed into a single fibroid; the other seven were salmonella-free.

“It probably spread through the gastrointestinal tract and thought ‘Here’s a nice nest for me,'” said Tachdjian, who practices in Santa Monica and is an associate clinical professor of medicine and pediatrics at the medical school UCLA David Geffen.

But the duration of Sternlieb’s infection, its location in a uterine fibroid, and the recurrent urticaria made this case a sort of fascinoma – medical slang for an unusual and unusually interesting case, a status that would be reinforced by the discovery of its source.

“I kept asking older doctors if they had ever seen anything like this, and they said no,” Schneider said. A search of medical journals conducted by Tachdjian revealed nothing similar.

As salmonella is a reportable disease, California health authorities have been informed.

Several months after his surgery, Sternlieb received a home visit from a public health nurse with surprising news: his infection had not been traced to food but to a reptile.

Turtles are known to harbor salmonella, one reason federal law has long banned the sale of small turtles because of the risk they pose to young children. Other reptiles, including snakes, frogs and lizards, also carry it. That’s why public health officials emphasize the importance of washing your hands after touching them.

But his family never had a pet reptile, Sternlieb said. Because her symptoms began shortly after giving birth, Sternlieb’s infectious disease doctor suspected she might have contracted the infection at the hospital, perhaps from a staff member. Sometimes during pregnancy and before delivery, the mother’s immune system is weakened to prevent her from rejecting the fetus.

Another possibility, said Sternlieb, who racked his brain trying to recall possible reptile exposures that occurred nearly two decades earlier, is that the infection was transmitted from a pet reptile to the The nursery school that her son, then aged 4, attended. But she said he never brought home a reptile and she didn’t remember the school keeping such pets.

Schneider said she began recovering almost immediately after the surgery and never had another episode. Doctors considered the operation a cure.

Tachdjian said he suspected she was exposed at the hospital and added it was fortunate she had surgery at that time. If the fibroid had ruptured, Sternlieb could have developed sepsis, a potentially fatal infection resulting from the spread of bacteria into the bloodstream.

In 2010, Tachdjian, Schneider and two other doctors published a report on her case in the journal Obstetrics and Gynecology. Their goal, Tachdjian said, was to alert other doctors to consider abdominal hives as a possible sign of a latent pelvic infection.

“You want these reports so the next (doctor) faced with something like this gets imaging quickly,” he said.

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