“I think this could be something serious,” Dr. Tore Kvaslerud explained quietly. “You need to go to the hospital.” The 30-year-old woman and her mother took in the information wordlessly. Although the news was bad, it was what they expected.
The rash started nearly two weeks earlier, as a little patch, maybe the size of a quarter on her forearm: red and bumpy and very itchy. Then it took over her entire body. She hardly recognized the bright red face that looked back at her from the mirror. Her clear, smooth skin was now irregular, lumpy and swollen, as if somehow inflated with air. Her lips were puffy and cracked; her ears huge and so tender that she couldn’t rest her head on her pillow. The slightest pressure was agony.
She went to the emergency room twice while she was staying with her mother in Franklin, Tenn., and both times was told that this was just a virus and it would pass. There was nothing to be done. Cold compresses might help her get through it, she was told, but it should get better on its own. The doctor she saw on her second visit terrified her when he told her that it could be acute H.I.V. But a blood test was done, and after hours of anxious waiting, the doctor returned to tell her that it was negative. It was probably just a virus. She should follow up with her primary-care doctor.
That’s when the young woman got in touch with Kvaslerud, the family-medicine doctor she had when she lived in Nashville, before she moved to New York City three years earlier. Thankfully, his office was able to make her an appointment that week.
Reasons for Extreme Concern
Kvaslerud was as warm and kind as she remembered. He studied her face as she explained what she had been through. It began with a sore throat and fever that lasted a couple of days, she told him. But then the rash started and simply hadn’t stopped.
The doctor kept asking questions as he examined her. She didn’t have a fever, but her heart was racing at 157 beats per minute. The lymph nodes in her neck were enlarged and tender. And her arms, legs and torso were covered with the same dramatic-looking rash that covered her face. There were scabs scattered across her body where she had scratched her skin raw.
The woman told Kvaslerud that she had been on a variety of antibiotics over the past few months for a bacterial infection that had been hard to treat. She lost her father the previous year and that had been hard; she recently started taking a medication, called Lamictal, to manage a depression that had not responded to other treatments.
Hearing the name of the drug, something clicked for Kvaslerud. It wasn’t a medication that he prescribed often, but he remembered that it could be associated with some severe skin reactions. He was immediately worried that this was Stevens-Johnson Syndrome, a rare skin disorder that is often triggered by medications like Lamictal, as well as by certain antibiotics. The syndrome causes the topmost layers of skin to separate, often producing large blisters that slough off, exposing the delicate dermis below. Patients had to be treated as if they had third-degree burns. And like burns, these denuding skin reactions could be deadly.
Kvaslerud’s office was in a complex linked to the Ascension Saint Thomas Hospital Midtown. He walked the patient and her mother over to the emergency room and explained to the doctor on duty what was going on. “I think she should be taken to Vanderbilt,” he told the physician, referring to the large academic medical center nearby. But the E.R. doctor was worried that she was too sick to travel even that far and admitted her to their intensive-care unit.
An Unusual Reaction
Dr. Allen Vantrease, the internist in charge of the patient’s care in the I.C.U., was also worried. Her heart rate remained over 150 beats per minute. Her white-blood-cell count was high, which can occur in infections.
Vantrease didn’t think she had Stevens-Johnson Syndrome. There was no evidence that her skin was actually peeling off. It didn’t look to him like any of her mucus membranes were damaged, which is usually the case with Stevens-Johnson. And the hugely swollen and tender lymph nodes she had in her neck were not characteristic of the syndrome.
There wasn’t a dermatologist on duty, so Vantrease texted Dr. Adrian O. Rodriguez, who taught him during his residency a decade before. Was this Stevens-Johnson Syndrome, as her primary-care doctor had thought, Vantrease asked Rodriguez, or was it another equally rare condition called drug reaction with eosinophilia and systemic symptoms, or DRESS? DRESS often started with a fever and sore throat and was followed by an extensive rash, facial swelling and injury to at least one internal organ system. DRESS was also potentially deadly, Vantrease knew from a patient he cared for years earlier.
Rodriguez agreed: This probably was DRESS. The most important step in caring for these very sick patients was to identify and stop the medication that started the whole thing. In this case, timing made the cause clear: Her most recently started medication, Lamictal, was one of the most common triggers of this rare but dangerous reaction. The treatment for DRESS was high-dose steroids to quell the inappropriate immune response. She was started on a steroid called methylprednisolone, along with an antihistamine. The patient could feel the itch and swelling start to relent after her first doses. She would have to remain on these medications for weeks, she was told.
A Long Recovery
She was discharged from the hospital after three days and given instructions on how to slowly wean off the steroid. She could continue the antihistamines for as long as she needed them. She followed the guidance closely and saw Rodriguez a few days after she got home.
It was then that Rodriguez ordered some follow-up lab tests, because she had some abnormalities while she was in the hospital. The new tests showed a severe injury to her liver. Rodriguez called her as soon as he got the results. The patient needed to go back to the hospital so they could figure out what was happening. A liver biopsy revealed that this damage was also a reaction to the Lamictal — a late component of DRESS.
Rodriguez was worried. He had seen this syndrome before, but this case was worse than the ones he had experience with. He wasn’t sure what to do next. He set out to find a doctor with more expertise. The patient did the same thing. She found Dr. Elizabeth Phillips, a doctor at Vanderbilt who studied DRESS.
When the patient arrived for her first appointment, Phillips noticed that she seemed strangely slow in her movements, in her speech and in her thinking. One of the many unusual characteristics of DRESS is that it can trigger autoimmune disorders. Phillips sent the young woman to the lab to get some blood work. As she expected, the patient’s thyroid gland was not working at all, most likely a casualty of her immune cells gone haywire.
The doctor started the patient on thyroid hormone replacement and continued the high-dose steroids. Slowly, the sluggishness receded and the swelling in her face improved. The bumpy rash gradually disappeared. She was able to inch her dose of steroid down over the course of many months.
Now, a year and a half later, she is mostly better. But DRESS has done some real damage to the young woman’s body. Her liver got worse before it got better. Her face is still red; she’s not sure it will ever regain its normal color or smoothness. She always had beautiful skin and she’s sad that she might never have it again. And her thyroid remains impaired. She’ll probably be on thyroid hormones for the rest of her life. She doesn’t really understand why Lamictal had such a horrible effect on her, but she is grateful that she found a doctor familiar with this strange and rare disorder.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write to her at [email protected].
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