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A 7-month-old girl with a febrile illness, rash
A 7-month-old girl was referred from the dermatology clinic for further evaluation of a febrile illness with a rash, with concern for Kawasaki disease.
The history of this illness began four weeks earlier with the onset of fever up to 104.7° F. At that time, she was noted to have a scarlatiniform rash and some diffuse, mildly painful swelling of the hands and feet. During this “acute stage,” no lab tests were done and she was diagnosed with a “viral syndrome” with otitis media and treated with amoxicillin. These symptoms lasted about a week, and then briefly resolved. Two days later, she began spiking fevers again, but this time the rash was more urticarial-like, with large, raised circular lesions without central clearing, scattered over the trunk and extremities. The rash would flare with fever and fade when her temperature came down. This time, she was diagnosed with erythema multiforme and persistent otitis media and treated with an injection of ceftriaxone and given a second-generation oral cephalosporin. As the fever and rash waxed and waned over the next week, the joint swelling returned. She was then referred to the dermatology clinic.
Pediatric Infectious Disease, Scott and White's Children's Health Center and Associate Professor of Pediatrics,
Texas A&M University, College of Medicine, Temple, Texas.
e-mail: jhbrien@aol.com
There was never any history of mucous membrane erythema or lymphadenopathy, but a complete blood count done in the pediatric clinic during the last clinic visit revealed a platelet count of 535,000 with a white blood cell count of 20,000 and an erythrocyte sedimentation rate (ESR) of 80.
Her past medical history was unremarkable and her immunizations were documented up to date. There had been no recent travel or insect exposure. She has contact with a family dog, but no other animal exposure.
Examination in the hospital revealed frequent fever spikes to 102° F. The rest of her vital signs were normal. She has had a one pound weight loss. She also had an evanescent pink rash with painful joint swelling as noted above, plus swelling of the knees, especially the right knee as shown in figures 1 – 4. Orthopedic surgery was consulted, and did a diagnostic aspiration of the right knee revealing 51,000 white blood cells with a predominance of segmented neutrophils and a normal glucose and negative Gram stain.
What’s your diagnosis?
- Measles
- Kawasaki disease
- Juvenile idiopathic arthritis
- Erythema multiforme
Answer
I first should say up front that I would not have recommended an arthrocentesis of her knee because there was nothing about her presentation that suggested septic arthritis, which I also left out of the list of choices.
This is a fairly typical presentation for C, juvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatoid arthritis (JRA). As with this case, the average time to diagnosis is about 30 days, and there are usually numerous other possibilities entertained before arriving at the diagnosis of JIA. The possibility of septic arthritis and toxic synovitis are entertained early on, but generally ruled out by the time a couple of weeks have passed. There are several subtypes of JIA. The type this child had is most likely “polyarticular rheumatoid factor-negative.” This is the second most common (20% to 30%) form of JIA, with 90% being in girls and can occur at any age, including infants. The joints involved are commonly hands, feet, hips and knees.
After confirmation of the diagnosis by pediatric rheumatology, the patient was initially treated with a combination of prednisone and ibuprofen, with fairly good, rapid results as shown in figures 5 – 7, taken when she was afebrile. However, as is the case in about 15% of patients with this type JIA, she developed severe disease, requiring more aggressive therapy with methotrexate.
There’s not enough information given to make the diagnosis of Kawasaki disease (KD) in the early, acute stage of this febrile illness. However, there were certainly enough findings present initially to be concerned for KD, and could be consistent with the incomplete form. The same rash may be seen in KD (Figure 8), as well as the swelling of the hands and feet (Figure 9). The absence of inflammation of the eyes or other mucous membranes and lymphadenopathy speak against KD, but are not necessary for the diagnosis of incomplete KD. By the time a month goes by, most patients with KD will be back to normal with or without treatment, unless they develop aneurysmal complications.
Measles causes a febrile exanthem (figure 10), along with conjunctivitis, coryza and cough, and a generalized ill feeling. While it may carry the risk for significant neurological sequelae, measles will also have run its course in much less than a month. Additionally, as uncommon as JIA might be, measles in this country is even less common.
Lastly, erythema multiforme (EM), a rash consisting of “fixed” lesions that are round, often resembling a target with dusky or necrotic centers (Figure 11), can be associated with fever, and can last for weeks and/or be recurrent, depending on the underlying cause. However, the polyarthritis is not likely to be seen with EM.
Columnist comments
Cases like the one above are eventually seen in all pediatric practices if you see enough patients over a long enough career. I began residency almost 31 years ago. I guess that makes me a PL-31 (I need a nap). During all that time, I can remember being involved in the diagnosis of about two patients per year; more since I have been in a hospital-based practice, since they usually end up being admitted for diagnosis. For that reason, I keep thinking that JIA is becoming more common, but this could simply be a sampling bias.
In most parts of the country, there are no local pediatric rheumatologists, who tend to be concentrated in the large, academic medical centers, and patients have to travel sometimes great distances in order to be evaluated for the best therapy (especially in Texas where, as in outer space, distance is measured by time rather than distance).
The primary should be the one treating the patient, but should be guided by an expert in these diseases. As this patient demonstrated, they are often resistant to conventional therapy, requiring more aggressive management with strong immune modulators. So, if you are a younger resident (less than a PL-31), and looking for a subspecialty, you would have a lot of work in the field of pediatric rheumatology. I doubt that it’s as much fun as infectious diseases, but it’s not a bad second choice.
Lastly, more than 4,000 soldiers have died in Iraq and Afghanistan since the war began. I fear that as public support for the war continues to fade, it may be natural to subconsciously lose support for the soldiers, airmen and sailors as well. As I have mentioned before, many of these selfless individuals are our colleagues in uniform. I was told at the Annual Uniformed Services Pediatric Seminar (USPS) in March that many pediatricians are on their Third Tour into one of these combat zones, filling a variety of jobs, demonstrating the versatility of pediatricians. Security restrictions prevented me from obtaining any details, but pediatrics as a specialty of deployed physicians is over-represented by the percentage of those being sent. In spite of this fact, their morale remains high. During the entire week at the meeting, I never heard a SINGLE WORD of dissent from any of the attendees. They all make me proud and honored to have been one of them at one shining moment in my life.
What’s Your Diagnosis? is a monthly case study featured in Infectious Diseases in Children, with treatment information and discussion to follow.