PFAPA syndrome: with regard to a case.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Hernández-Bou
S
S
Sección Inmunoalergia. Unidad Integrada de Pediatría Hospital Clínic-Hospital Sant Joan de Déu. Universidad de Barcelona, Spain.
Giner
M
M
Plaza
A M
AM
Sierra
J I
JI
Martín Mateos
M A
MA
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis; D005260:Female; D005334:Fever / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007239:Infections / Q000175:diagnosis; D008206:Lymphatic Diseases / Q000188:drug therapy / Q000209:etiology*; D010507:Periodicity; D010612:Pharyngitis / Q000188:drug therapy / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D013281:Stomatitis, Aphthous / Q000188:drug therapy / Q000209:etiology*; D013577:Syndrome; D017211:Treatment Failure
D000893:Anti-Inflammatory Agents; D011241:Prednisone