2016, Number 4
An Med Asoc Med Hosp ABC 2016; 61 (4)
Rodríguez WLO, Betech RA, Pineda BLG, Ortiz ROE
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ABSTRACTWe report a representative case of periodic fever. PFAPA syndrome is an entity consisting of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. It is an autoinflammatory disease whose etiology is unknown. It is the most common cause of periodic fever in childhood. It presents between two and five years of age and is more common in males. Fever episodes may last three to six days, with an interval of recurrence between two and eight weeks. The syndrome includes aphthous stomatitis seen in the vestibule of the mouth, non-exudative pharyngitis and anterior adenitis which disappear with the remission of the fever. Acute phase reactants are elevated and leukocytosis may occur with neutrophilia. PFAPA is an exclusion diagnosis and it is a self-limited condition. Effective treatments for PFAPA syndrome are oral steroids; in some cases, we should consider adenotonsillectomy.