A yoga and meditation teacher who worked in the pediatric pain program at UCLA, Sternlieb underwent numerous tests that failed to reveal the cause of the unidentified ailment, which occurred two or three times a year and lasted about five days . In 2004, after 17 years, the disease began to occur 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 finally identified after Sternlieb underwent surgery that eventually cured her.
“It was good that my belly turned red because it got the attention of my doctors,” Sternlieb said recently. “Something was really wrong, but no one imagined this.”
The first episode occurred in December 1987, two weeks after the birth of Sternlieb’s second child. “I became sicker than ever,” said Sternlieb, then 37. “It was flu season and it was a bad flu season this year,” so doctors attributed her illness to the flu.
Six months later, the disease recurred, a pattern that prevailed for years.
At first, Sternlieb didn’t pay much attention to the small red dots that covered her stomach. The rash looked like a sunburn, but it wasn’t itchy or painful. Doctors eventually decided it was hives, a common skin condition that can occur as an allergic reaction to food or medicine; often the cause is never discovered.
“I became sicker than ever. … It was flu season and it was a bad flu season this year,” so doctors attributed her illness to the flu.
— Beth Sternlieb
Her primary care physician referred her to a rheumatologist, a doctor who specializes in the treatment of autoimmune diseases, whom she had been seeing for several years. He ordered blood tests, which he said suggested an unspecified autoimmune disease in which the body mistakenly attacks itself.
Over the years, Sternlieb has noticed that episodes seem to occur during periods of stress “both good and bad,” including travel, partying and too little sleep. “I thought there had to be a psychological component,” she said.
She learned to include the attacks in her life, relieved that no one had found anything serious. She hoped the doctors would figure out what was wrong so they could treat and eradicate whatever it was.
In 2005, Sternlieb’s peace was shattered by the sharp deterioration of her health.
That summer she fell seriously ill and never recovered. Her fever periodically rose to 104 degrees and she suffered from profuse night sweats as well as severe weakness and fatigue. She lost 15 kilograms and, unable to work, spent most of her time in bed or on the couch. The rash, which was confined to her abdomen, spread to her neck and torso. Blood tests showed increased levels of inflammation and a high white blood cell count.
Sternlieb began visiting a new group of specialists. An infectious disease doctor reviewed her travel history, which included a trip to India years earlier, ultimately ruling out malaria and other parasitic infections. Doctors considered and ruled out various diagnoses, including a fever of unknown origin that could be related to some autoimmune diseases; familial Mediterranean fever, an inherited genetic disorder that causes recurring fevers and inflammation; as well as HIV and hepatitis.
That left infection or allergy as possible causes. The latter seemed unlikely despite the recurring hives, said Rafi Tachjian, then a fellow in allergy and immunology at UCLA and one of the doctors Sternlieb consulted.
“The rashes usually last 24 hours and are not like those that were chronic,” he recalls. “We had to look deeper into anything unusual… It seemed like there was something collecting a little heat somewhere,” prompting a reaction from Sternlieb’s Immune System.
“We see this in the sinuses, where the antibiotics don’t reach the infected tissue” and lead to a smoldering 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 do not require treatment unless they cause problems. The scan showed that one of the fibroids had grown a lot and was probably degenerating (dying) or necrotizing (dead), which happens when the tumor loses its blood supply.
A degenerating fibroid can become very large very quickly. But doctors were also concerned about the possibility of a rare cancer like leiomyosarcoma, which grows in smooth muscle, including 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 the hives that are not related to fibroids or this cancer?
“It didn’t seem obvious that the fibroid would cause this,” said Schneider, of Cedars-Sinai Medical Group. “But it didn’t look like a typical fibroid, and I recommended we take it out.” Sternlieb, who said she was worried she might still be sick after the hysterectomy, agreed.
During the operation in December 2005, Schneider removed eight fibroids. The largest was 11 centimeters, the size of a large grapefruit.
Nearly 20 years later, Schneider clearly remembers its unique characteristics. Usually, a fibroid is a hard ball of muscle, she said. This one was full of pus, which erupted explosively when touched with a scalpel.
“It was crazy,” said Schneider, who had never seen anything like it before and hasn’t since. She administered antibiotics and took a culture, which she sent to the pathology lab for analysis.
Tachjian remembers Schneider calling him right after the surgery was over to tell him what she had found.
“I thought we should know what the hell grew up,” Tachjian said. “We’re crossing our fingers that the surgery takes care of it, whatever it is.” But only time will tell.”
A few weeks later, the first question was answered. The culture showed 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 annually and results in more than 26,000 hospitalizations and 420 deaths. Neither Sternlieb nor her doctors knew how or when she contracted salmonella, which is known to cause hives if it settles in the intestines, Tachjian said.
In Sternlieb’s case, the bacterium burrowed into only one fibroid; the remaining seven were free of salmonella.
“I kept asking older doctors if they had ever seen anything like this and they said no.”
— Jessica Schneider, gynecologist
“It probably planted itself in the gastrointestinal tract and thought, ‘Here’s a nice nest for me,'” said Tachjian, who practices in Santa Monica and is an associate clinical professor of medicine and pediatrics at the UCLA David Geffen School of Medicine.
But the duration of Sternlieb’s infection, its location in the uterine fibroid, and the recurrent urticaria made the case something of a fascinoma—medical jargon for an unusual and unusually interesting case, a status that would be enhanced by the discovery of its source.
“I kept asking older doctors if they’d ever seen anything like this, and they said they hadn’t,” Schneider said. A search of medical journals by Tachjian turned up nothing of the sort.
Because salmonella is a reportable disease, California health officials were notified.
A few months after the operation, Sternlieb received a home visit from a public health nurse with startling news: her infection was not traced to food, but to a reptile.
Turtles are known to contain salmonella, one of the reasons federal law has long prohibited the sale of baby turtles because of the risk they pose to young children. Other reptiles, including snakes, frogs and lizards, are also carriers, which is why public health officials stress the importance of washing hands after touching them.
But her family never had a pet reptile, Sternlieb said. Because her symptoms began shortly after birth, Sternlieb’s infectious disease doctor suspected she may have contracted the infection at the hospital, possibly from a staff member. Sometimes during pregnancy and before birth, the mother’s immune system is suppressed to prevent rejection of the fetus.
Another possibility, said Sternlieb, who wracked her brain trying to recall possible reptile exposures from nearly two decades earlier, is that the infection was transmitted by a pet reptile at the daycare her then 4-year-old son attended. But she added that he had never brought a reptile home and that she did not recall the school keeping such pets.
Schneider said she began to recover almost immediately after the surgery and has never had another episode. The doctors believe that the operation is a cure.
Tachjian said he suspects she was exposed at the hospital and added it was lucky she had surgery when she did. If the fibroid had ruptured, Sternlieb could have developed sepsis, a potentially fatal infection that results from bacteria traveling through the bloodstream.
In 2010, Tachdjian, Schneider and two other doctors published a report on her case in the journal Obstetrics and Gynecology. Their goal, Tachjian said, was to warn other doctors to consider hives as a possible sign of a smoldering pelvic infection.
“You want these reports, so the next one [doctor] who comes across something like this is quickly visualized,” he said.
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