A 5-year-old girl with no relevant personal history.
For several months she has had recurrent episodes of high fever (every 4 weeks), laterocervical lymphadenopathy and pharyngitis.
Episodes are characterized by fever (39oC) lasting 3-4 days, with little response to antipyretics.
The child is asymptomatic between episodes and has a normal development for her age.
Laboratory tests showed leukocytosis with moderate neutrophilia, as well as elevated erythrocyte sedimentation rate and C-reactive protein (CRP).
Pharyngeal smears were negative on several occasions.
The first episodes were treated with antibiotics.
There was no improvement until day 3.
Described symptoms, their recurrence and negative pharyngeal cultures, which occurred repeatedly, were thought of the possibility of Marshall syndrome.
Coinciding with a new febrile peak, oral corticosteroid therapy was initiated.
The episode lasted less than 6 hours after taking prednisone.
This response was tested in subsequent episodes, suggesting a PFAPA syndrome.
