The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.

Rationale: Kawasaki disease (KD) is an acute vasculitis of small and medium vessels; whereas systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease. Their presentation is varied and not always straightforward, leading to misdiagnosis. There have been case reports of lupus onset mimicking KD and KD presenting as lupus-like. Coexistence of both diseases is also possible. Patient concerns: We present three adolescents, one with fever, rash, arthritis, nephritis, lymphopenia, and coronary aneurysms, a second patient with rash, fever, aseptic meningitis, and seizures, and a third patient with fever, rash, and pleural effusion. Diagnoses: The first patient was finally diagnosed with SLE and KD, the second patient initially diagnosed as KD but eventually SLE and the third patient was diagnosed at onset as lupus but finally diagnosed as KD. Interventions: The first patient was treated with IVIG, corticosteroids, aspirin, coumadin and mycophenolate mofetil. The second patient was treated with IVIG, corticosteroids and methotrexate and the third patient with IVIG, aspirin and corticosteroids. Lessons: Both diseases may mimic each other's clinical presentation. KD in adolescence presents with atypical signs, incomplete presentation, and develop coronary complications more commonly. An adolescent with fever and rash should include KD and SLE in the differential diagnosis.

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Though the causative agent of Kawasaki disese (KD) remains unclear, an infectious agent is thought to trigger the disease. KD has been described associated with immunodeficiency disorders including HIV. We report a patient with Acute Myeloid Leukemia (AML) who developed KD in an early course of the disease. A 4 year-old boy was diagnosed as AML and received treatment with daunorubicin, etoposide and Ara-C. Eleven days later he started presenting fever, diarrhea and abdominal pain. A diagnosis of neutropenic colitis was made and treatment with vancomycin and cefepime was started. He continued having fever and polymorphic eruption was added, with prominent perineal erythema. Later he presented redness of the lips, hands and feet edema, erythema in the palms and soles and conjuctival erythema. The diagnosis of KD was established and treatment with IVIG and low-dose prednisone was administered. Echocardiogram revealed left coronary aneurysm and pericardial effusion. Abdominal-ultrasound showed gallbladder hydrops. Candida grew in blood culture and treatment with amphotericin-B was started. Akita et al. reported one previous case of leukemia associated with KD. As the previous reported case, our case developed the disease in the induction therapy for the AML. Interestingly, infection with Candida was documented in our patient. Administration of Candida cell Wall-antigens induced KD-like coronary vasculitis in mice. C. abicans colonizes the intestinal tract and causes invasive deep micosis in an immune-compromised host. Beta-glucan from the fungus stimulates the host immune system inducing an inflammatory response. Sampling campaigns over Japan during the KD season detected major differences in the microbiota of the tropospheric aerosols, with the unexpected finding of the Candida species as the dominant fungus from aloft samples (54% of all fungal strains). These results, provide support for the feasibility of a windborne pathogen. A fungal toxin could be pursued as a possible etiologic agent of KD. To our knowledge this is the second case of KD associated with leukemia. Chemotherapy-induced immunosuppresion could have a role in the genesis of KD in our patient and possibly triggered by a Candida infection.

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