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Wikenigma - an Encyclopedia of Unknowns Wikenigma - an Encyclopedia of the Unknown Wikenigma - an Encyclopaedia of Unknowns Wikenigma - an Encyclopaedia of the Unknown


“Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a medical condition, typically starting in young children, in which high fever occurs periodically at intervals of about 3–5 weeks, frequently accompanied by aphthous-like ulcers, pharyngitis and/or cervical adenitis (cervical lymphadenopathy). The syndrome was described in 1987 and named two years later.”

Source: Wikipedia

PFAPA is the most common periodic fever syndrome in children, but its cause(s) and its pathogenesis are currently unknown. Suggested possible causes include problematic genetics and/or effects of previous infection.

Research from 2020 suggests that two other diseases, aphthous stomatitis and Behçet’s disease may be on a 'common spectrum' along with PFAPA - and could be different manifestations of the same 'spectrum disorder'.

See: Common genetic susceptibility loci link PFAPA syndrome, Behçet’s disease, and recurrent aphthous stomatitis

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