Kawasaki disease in a PFAPA patient: should be aware of this combination

Konstandinos Diakolios, Evdoxia Bourazani, Alkistis Adramerina, Konstandina Machairidou, Charalambos Neocleous


Objective – In publishing this case we aim to support the primary association between Kawasaki Disease and periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) in children, a position that previously suggested by Ninomiya et al. (Development of Kawasaki disease in a patient with PFAPA. Pediatr Int. 2013; 55:801-2).

Case report – A case of a 24-month-old boy with known PFAPA syndrome who developed Kawasaki disease is presented. Our case shared many similarities with the patient treated by Ninomiya et al. and supports their opinion that there might be a primary association between Kawasaki Disease and PFAPA syndrome in children.

Conclusion – We suggest that there must be an awareness of this combination. Awareness of this association can lead the prompt diagnosis of Kawasaki disease and early treatment to reduce the sequelae.


Kawasaki; PFAPA

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