Medical Mysteries: Years of hives and fevers traced to a startling cause
A California woman suffered from an episodic flu-like illness that defied explanation. Its origin stunned her doctors.
A yoga and meditation teacher who worked at the University of California at Los Angeles’s pediatric pain program, Sternlieb had undergone numerous tests that failed to reveal the reason for the unidentified illness that occurred two or three times a year and lasted about five days. In 2004, after 17 years, the illness began occurring 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 startling and highly unusual cause was finally identified after Sternlieb underwent an operation that ended up curing her.
“It was a good thing my abdomen turned red because it caught the attention of my doctors,” Sternlieb said recently. “Something was really wrong, but no one imagined this.”
Bad case of flu
The first episode occurred in December 1987, two weeks after Sternlieb’s second child was born. “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 her illness to influenza.
Six months later the illness recurred, a pattern that prevailed for years.
At first Sternlieb didn’t pay much attention to the tiny red dots that covered her abdomen. The rash resembled a sunburn but wasn’t itchy or painful. Doctors ultimately decided it was hives, a common skin condition that can occur as an allergic reaction to food or drugs; often its cause is never discovered.
“I became sicker than I had ever been. ... It was flu season and that year was a bad flu season,” so doctors attributed her illness to influenza.— Beth Sternlieb
Her primary care doctor referred her to a rheumatologist, a physician who specializes in treating autoimmune diseases, whom she saw for several years. He ordered blood tests, which he said suggested the presence of an unspecified autoimmune disorder in which the body mistakenly attacks itself.
Over the years, Sternlieb noticed that the episodes seemed to occur during periods of stress “both good and bad,” including traveling, partying and too little sleep. “I thought it must have a psychological component,” she said.
She learned to incorporate the bouts into her life, relieved that no one had found anything serious. She hoped doctors would figure out what was wrong so they could treat and eradicate whatever it was.
Travel history
By 2005, Sternlieb’s equanimity was shattered by a sharp deterioration in her health.
That summer, she became seriously ill and did not recover. Her fever periodically spiked to 104 degrees, and she suffered from drenching night sweats as well as 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 had been confined to her abdomen spread to her neck and torso. Blood tests showed elevated levels of inflammation and a high white blood cell count.
Sternlieb began seeing a new crop of specialists. An infectious-disease doctor combed through her travel history, which included a trip to India years earlier, ultimately ruling out malaria and other parasitic infections. Doctors considered and discarded a variety of diagnoses including fever of unknown origin, which can be associated with some autoimmune diseases; familial Mediterranean fever, an inherited genetic disorder that causes recurrent fevers and inflammation; as well as HIV and hepatitis.
That left an infection or an allergy as possible causes. The latter seemed unlikely despite recurrent hives, said Raffi Tachdjian, then a fellow in allergy and immunology at UCLA and one of the doctors Sternlieb consulted.
“Hives usually last 24 hours and are not like this, which was chronic," he recalled. “We needed to look deeper into anything unusual....It seemed that there was something packing some heat somewhere” that was triggering a reaction from Sternlieb’s immune system.
“We see this in sinuses where antibiotics don’t reach infected tissue” and result in a smoldering infection that becomes virtually impossible to eradicate with medication, he added.
A CT scan ordered by the infectious-disease doctor showed multiple uterine fibroids, common benign tumors that don’t require treatment unless they cause problems. The scan showed that one of the fibroids had grown very large and was possibly degenerating (dying) or necrotic (dead), which happens when a tumor loses its blood supply.
A degenerating fibroid can get very big very fast. But doctors were also concerned about the possibility of a rare cancer such as a leiomyosarcoma, which grows in smooth muscles including in uterine tissue. None of her doctors, including her new gynecologist Jessica Schneider, knew whether her long-standing illness and the fibroids were related.
And what explained the hives, which are not associated with fibroids or this cancer?
“It didn’t seem obvious that a fibroid would cause this,” said Schneider, a member of the Cedars-Sinai Medical Group. “But it didn’t look like a typical fibroid, and I recommended we take it out.” Sternlieb, who said she worried she might still be sick after a hysterectomy, agreed.
During the December 2005 operation, Schneider removed eight fibroids. The largest measured a whopping 11 centimeters, the size of a large grapefruit.
Nearly 20 years later Schneider vividly remembers its unique characteristics. Typically a fibroid is a solid ball of muscle, she said. This one was full of pus that spurted explosively when it was 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 that she sent to the pathology lab for analysis.
Tachdjian remembers that Schneider called him just after she finished surgery to tell him what she had found.
“I thought ‘We need to know what the heck grew,’ ” Tachdjian said. “We were crossing our fingers that surgery would take care of it, whatever it was. But only time would tell.”
‘A nice nest’
A few weeks later, the first question was answered. The culture showed an unknown strain of salmonella, a common bacterial infection that is 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 intestine, Tachdjian said.
In Sternlieb’s case, the bacteria had burrowed into only one fibroid; the other seven were free of salmonella.
“I kept asking older doctors if they’d ever seen anything like this, and they said they hadn’t.”— Jessica Schneider, gynecologist
“It probably seeded itself in the GI 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 UCLA David Geffen School of Medicine.
But the duration of Sternlieb’s infection, its location in a uterine fibroid and the recurring hives made the case something of a fascinoma — medical slang 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 conducted by Tachdjian turned up nothing similar.
Because salmonella is a reportable disease, California health officials were notified.
Several months after her operation, Sternlieb received a home visit from a public health nurse with startling news: Her infection had been traced not 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 are also carriers, which is why public health officials stress the importance of handwashing after touching them.
But her family never had a pet reptile, Sternlieb said. Because her symptoms started shortly after giving birth, Sternlieb’s infectious-disease doctor suspected she might have contracted the infection in the hospital, possibly from a staff member. At times during pregnancy and before delivery, the mother’s immune system is suppressed to prevent it from rejecting 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 nursery school her then-4-year-old son attended. But she added that he never brought a reptile home and that she doesn’t remember the school keeping such pets.
Schneider said she began to recover almost immediately after surgery and has never had another episode. Doctors deemed the surgery a cure.
Tachdjian said he suspects she was exposed in the hospital and added that it’s fortunate she underwent surgery when she did. Had the fibroid ruptured, Sternlieb could have developed sepsis, a potentially fatal infection that results from bacteria coursing through the bloodstream.
In 2010, Tachdjian, Schneider and two other doctors published a report of 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 simmering pelvic infection.
“You want these reports so the next [doctor] that runs into something like this gets imaging quickly,” he said.
Send your solved medical mystery to sandra.boodman@washpost.com. No unsolved cases, please. Read previous mysteries at wapo.st/medicalmysteries.
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