Hernández-Bou S, Giner M, Plaza A M, Sierra J I, Martín Mateos M A
Sección Inmunoalergia. Unidad Integrada de Pediatría Hospital Clínic-Hospital Sant Joan de Déu. Universidad de Barcelona, Spain.
Allergol Immunopathol (Madr). 2003 Jul-Aug;31(4):236-9. doi: 10.1016/s0301-0546(03)79185-x.
PFAPA syndrome (Periodic Fever, Aphtas, Pharyngitis and cervical Adenopathies) is one of the causes of periodic fever in pediatrics and it is characterised by high fever, pharyngitis, cervical adenitis and aphtous stomatitis. Its etiopathogeny is unknown. The diagnosis is clinical and the findings of laboratory are unspecified. One or two doses (1 mg/kg) of oral prednisone are enough for a fast resolution of the clinic. It is a benign syndrome and no sequels have been noticed after its disappearance, usually in four years from its beginning.
We present the case of a 10-year-old patient who has been diagnosed of PFAPA syndrome after 3 years and a half of characteristic clinical bouts, with the fulfilment of diagnostic criteria and after having excluded other entities of similar presentation.
Periodic episodes of high fever, pharyngitis and cervical adenitis with a bad response to the conventional treatment should alert us to the PFAPA syndrome. The recognition of this entity will help us to improve the diagnostic and therapeutical focusing, lowering also the anxiety that these cases produce.
