Síndrome PFAPAde la sospecha al diagnóstico

Abstract

Fever is one of the most frequent reasons for medical consultation during childhood. Usually is related to minor viral infections, but when lasts over time or episodes recur, it is necessary to make differential diagnosis of unusual infections, autoimmune diseases, immunodeficiencies and neoplasms. The PFAPA syndrome (periodic fever, pharyngitis, aphthous stomatitis and adenitis) belongs to the group of periodic fever syndromes characterised by episodes of fever that recur regularly for several months, alternating with healthy periods. It is the most common recurrent periodic fever disorder described in childhood and the clinical manifestations of PFAPA syndrome episodes are well known. Corticoids are the treatment of choice and resolve the febrile episode, and this spectacular outcome is used as diagnostic criterion. Early diagnosis and appropriate treatment can significantly improve the quality of life of both patients and families, and prevent drug toxicity. We present two preschool girls with PFAPA syndrome.