Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome

Periodic fevers (fevers that occur predictably at fixed intervals) are unus ual in infants and children. The classic periodic fever syndrome is cyclic neutropenia (neutropenia followed by infections and fever that recur every 21 days). A new periodic fever syndrome PFAPA (periodic fever, aphthous sto matitis, pharyngitis, and cervical adenitis) has been characterized over th e past decade. PFAPA is defined clinically, because specific laboratory abn ormalities have not been found. The clinical characteristic of PFAPA is hig h fevers (usually 40.0 degrees C to 40.6 degrees C) recurring at fixed inte rvals every 2 to 8 weeks. The fevers last for about 4 days, then resolve sp ontaneously. Associated with the fevers are aphthous stomatitis in 70% of p atients, pharyngitis in 72% of patients, and cervical adenitis in 88% of pa tients. PFAPA is not familiar and begins before the age of 5 years. An epis ode of PFAPA can be aborted with one or two small doses of prednisone. The episodes of PFAPA may last for years and the patient is well between episod es. The cause of PFAPA is unknown and there are no reported sequelae. Curr Opin Pediatr 2000 12:253-256 (C) 2000 Lippincott Williams & Wilkins, Inc.