PFAPA: Periodic Fever, Aphthous Ulceration, Pharyngitis, Adenitis

Abstract

PFAPA syndrome—Periodic fever with aphthous stomatitis, pharyngitis, and adenitis—is the most common auto inflammatory condition amongst children worldwide—with the caveat that excludes specific geographic regions with a high prevalence of Familial Mediterranean Fever. A cardinal feature in addition to the described clinical signs is the clockwork like regular periodicity of the fevers. In contrast to most of the other auto inflammatory conditions and cyclic neutropenia the course is usually benign, the condition is self-limiting and the long-term outlook is very favourable, with most children outgrowing the condition by 10 years of age. The precise aetiology remains unknown and to date no specific genetic mutation has been identified although familial cases have been described.