Rare autoimmune disorder stumps PCPs

“Please bring her up the back stairs, directly to my office,” I told the caller and then immediately contacted the county health department.  “Do we have measles?” I asked.

Assured that there were no cases in the past two years, I waited for my patient to arrive.  The family of three was a recent newcomer to my family practice who had been in for routine physical exams and immunizations. They had not yet met my partner/supervisory physician.

Four year old, “Amy,” arrived with her worried mother. The mother reported that Amy had developed a rash during the night with some restlessness and a temperature reaching 104° F that morning.  The child did not appear very sick, only somewhat subdued.  Upon questioning she reported to a sore throat, loss of appetite and mild fatigue.

Amy had a diffuse, fine, sandpaper-like strawberry-colored rash. Her temperature was 104.3° F. Other vital signs were within normal limits for her age, her chest was clear and she exhibited a regular heart rate and rhythm with no murmurs, gallops or rub.  Her tonsillar pillars were engorged and injected, and all cervical nodes were enlarged. Her abdomen was non-tender without organomegaly; the remainder of her exam was normal.

A quick strep test of the oropharyngeal swab was positive. Amy was diagnosed with strep throat and sent home with a prescription for penicillin and instructions to receive acetaminophen every four hours, as well as daily oral probiotics throughout the antibiotics course. Her mother was instructed to call at any time.

The following morning Amy's mother called describing an unusual response to the medication – when the fever responded to each dose of acetaminophen the rash also disappeared; three hours after each dose was administered both the fever and rash reappeared. I asked them to return to the office that morning and not to take the acetaminophen before the visit.

There was no change in the physical findings other then some improvement in the tonsillar engorgement. I left them in my office and found my partner in the hallway. “This is when the PA comes to her supervisory physician and says ‘Help',” I told him.  

But neither of us could figure it out, so we referred her to a pediatrician who saw the child the same day. The diagnosis was Kawasaki disease ­– almost unheard of in Caucasian children on the West coast.

Interestingly, there is some association between recent carpet cleaning and this autoimmune disease that affects children, mostly those aged younger than 5 years, and that can be fatal if left untreated. A professional upholsterer had cleaned the family's couch just two weeks prior to the onset of Amy's illness.

Amy and her mother were hospitalized in isolation for four days and were prescribed IV antibiotics, followed by six weeks of home quarantine to prevent the introduction of potential infectious organisms until her immune system recovered. Amy's father had to live with friends during the period, so he could go to work. Amy recovered fully without compromise, and we all learned from the experience.

 Tell us about your most memorable patient by December 15, 2010, and you'll be eligible to win an Apple iPad!

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