Presentation
A 10-year-old Hispanic girl presents with a history of recurrent fevers (temperature
up to 104.0°F [40.0°C]) over the past 4 years. She has had 4 to 5 episodes of fever,
each lasting 1 to 2 weeks. The episodes are accompanied by chills, malaise, periorbital
swelling, rash, myalgias, arthralgias, and diffuse abdominal pain. She denies
weight loss, night sweats, and oral or genital ulcers. Medical and family histories
are noncontributory.
Physical examination reveals a well-nourished girl who has a temperature of 102.2°F
(39.0°C), heart rate of 130 beats/min, respiratory rate of 20 breaths/min, and blood
pressure of 101/64 mm Hg. Erythematous, nonpruritic, polymorphous, blanching
maculopapular lesions of 0.5 to 3 cm in size are apparent on her trunk and upper
extremities. The rest of her physical findings are normal.
Her hemoglobin level is 10.7 g/dL (107 g/L), white blood cell count is 13.24 × 103/mcL
(13.24 × 109/L) (54% neutrophils, 2% bands, 30% lymphocytes, 14% monocytes),
and platelet count is 424 × 103/mcL (424 × 109/L). Her C-reactive protein value is
15.1 mg/dL (143.8 nmol/L), and erythrocyte sedimentation rate is 82 mm/h. Results
of her liver function tests, pancreatic enzymes panel, urine protein to creatinine
ratio, and serum complement assessments are normal. The antinuclear antibody,
antibody to double-stranded DNA, antinuclear cytoplasmic antibody, and direct
Coombs tests show negative results. Quantitative serum immunoglobulin concentrations,
including serum IgD, are normal. A computed tomography scan of the
abdomen and pelvis is read as normal. Additional laboratory evaluation establishes
the diagnosis.
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