Kawasaki Disease (KD) is one of the most common vasculitis of childhood. Patients with fever that last more than 5 days and fewer than four principal mucocutaneous inflammatory clinical features can be described as “atypical” or “incomplete” Kawasaki disease. The febrile phase of the disease is characterized by systemic inflammation in numerous organs, tissues, and medium-sized arteries, and aseptic meningoencephalitis may occur due to meningeal inflammation. Intravenous immunoglobulin (IVIG) -resistant KD is described as recrudescent or persistent fever at least 36 hours following completion of the first dose of IVIG.
A 5 year-5 month-old girl applied to our clinic with the symptoms of meningoencephalitis. She was diagnosed with incomplet KD, and recieved IVIG treatment. The patient had recrudescent fever and recieved a second dose of IVIG infusion. She then recieved steroid treatment in consequence of persistent clinical manifestations. As a result, significant clinical and laboratory improvement was detected.
There are many studies in the literature reporting IVIG-resistant KD. However,there are limited studies on IVIGresistant incomplete KD patients which presents with meningoencephalitis and responds to steroid treatment.Kawasaki disease should be considered in all febrile patients lasting longer than 5 days considering the atypical cases with central nervous system involvement.
Children; Incomplete Kawasaki disease; Aseptic meningoencephalitis