Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A Tunisian report

Summary 

PFAPA syndrome is a rare entity characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. The authors report a case with PFAPA syndrome. A 7-year-old girl had recurrent pharyngitis and aphthous stomatitis since the age of 6 years. She was hospitalized in orthopaedic care because of coxalgia. A septic arthritis was excluded. She was subsequently admitted in paediatric unit because of fever, chills and vomits. At physical examination, we found a feverish child having a pharyngitis and cervical adenitis. Laboratory analysis showed signs of inflammation. The girl was treated by azithromycin. One month later, she developed an aphthous stomatitis, fever and painful cervical lymphadenopathy treated by a single dose of prednisone (2mg/kg). She was diagnosed as having PFAPA syndrome. Her complaints dramatically and completely disappeared after administration of the drug. During the 10 months of follow-up, two similar attacks were noted. She was treated by a single dose of steroids. After the second relapse, she was put under cimetidine (40mg/(kgday)) with a good outcome.

Keywords: Periodic fever, Pharyngitis, Aphthous stomatitis, Marshall syndrome, PFAPA