Periodic fever accompanied by aphthous stomatitis, pharyngitis and/or cervical adenitis (PFAPA) was first described in 1987 by Marshall et al. Fever periodicity is a hallmark of the diagnosis, and there is a noted absence of upper respiratory tract (URTI) symptoms and sick contacts. Diagnosing PFAPA can be challenging for clinicians. The most common treatment is corticosteroids, given at the first sign of fever. A single dose of prednisone will abort the febrile episode; this is unique to PFAPA and essentially diagnostic.1 2 Tonsillectomy has also been reported to be curative.2
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