Abstract

Childhood-onset systemic lupus erythematosus (cSLE) may rarely present initially with features mimicking Kawasaki disease (KD). Here, we report a 10-year-old girl presenting with high-grade fever, generalized skin rash, conjunctivitis, cervical lymphadenopathy, dry fissured lips, strawberry tongue, polyarthritis, abdominal pain, and neck stiffness. She was initially diagnosed and treated as KD, and the diagnosis of cSLE was confirmed 4 weeks later when she presented with a malar rash, nasal ulcer, seizures, and polyarthritis. In addition, her antinuclear antibodies, anti-double-stranded DNA, and direct Coombs test were positive. Therefore, with the difficulties in differential diagnosis and the rarity of such presentation, children with KD who develop new or unexpected features need to be evaluated for the development of cSLE.

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