Abstract
Although the etiology of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome remain unclear, various treatments have been reported. We here describe two thought-provoking cases. Our experience suggests that tonsillectomy is effective against PFAPA syndrome, and can safely be performed in patients three years and older. We recommend that tonsillectomy be considered as early as possible in patients with PFAPA syndrome.
Highlights
Aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever lasting three to six days and recurring regularly every two to eight weeks
The fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis[1,2,3,4]
For safety reasons, tonsillectomy is generally recommended for patients aged three years and older[13], whereas oral medications can be used in younger patients
Summary
Aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever lasting three to six days and recurring regularly every two to eight weeks. Case 1: A six-years-age boy with an unremarkable medical history experienced periodic febrile attacks accompanied by aphthous stomatitis, pharyngitis, and cough every two to eight weeks (Table 1) He was diagnosed with PFAPA syndrome in accordance with the diagnostic criteria advocated by Thomas et al.[3] at three years of age, the onset of disease was at one year of age. Case 2: A three-years-age girl with an unremarkable medical history experienced periodic febrile attacks accompanied by aphthous stomatitis and pharyngitis every two to seven weeks (Table 1) She was diagnosed with PFAPA syndrome at the age of one year in accordance with the criteria of Thomas et al.[3] During febrile attacks, prednisolone (a single dose of 0.5 mg/kg, repeated in 12 hours if necessary, depending on response to treatment) effectively reduced the fever. Pathological examination in both cases revealed the findings of chronic tonsillitis, including enlarged and hyperplastic lymphoid follicles (Data not shown)
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