Development of Kawasaki disease in a patient with PFAPA

摘要: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases unknown etiology characterized by regularly recurrent fever episodes with attacks lasting 3-6 days every 3-8 weeks associated at least three cardinal clinical signs: pharyngitis, adenitis. Kawasaki disease (KD) an acute, self-limited systemic vasculitis that occurs predominantly in infants young children. In most KD patients, i.v. immunoglobulin leads to a rapid amelioration symptoms significantly decreases risk coronary artery aneurysms. Although still unknown, it was reported innate immunity activated patients. Described herein patient PFAPA who developed KD. This first report development patient. The association between may represent genetic predisposition dysregulated immune response.