PFAPA Syndrome and Hereditary Periodic Fever Syndromes

The periodic fever sydromes are a group of disorders characterized by recurrent fever episodes and localized inflammation. One of these, the PFAPA syndrome, is seen in childhood with 3-7 day episodes of fever that is often accompanied by aphthous stomatitis, pharyngitis and cervical lymphadenopathy. The child is usually asymptomatic between episodes. PFAPA sydrome is more frequent in children under five years of age in males and most of the time these patients have been diagnosed as having an upper respiratory tract infection and to be treated with antibiotics. We present four and eight years old patients with a long term diagnosis of recurrent tonsillitis who received penicillin and antipyretic treatment. Patients who were diagnosed with PFAPA syndrome were administered 1 mg/kg prednisolone and children responded very quickly to steroid therapy. Pediatricians and otorhinolaryngologists should consider this syndrome especially in rhythmic recurring fever attacks and antibiotic resistant tonsillitis in children. We presented two cases of PFAPA syndromes and reviewed periodic fever syndromes.