Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

Unknown-2This syndrome includes recurrent episodes of fever with aphthous stomatitis (mouth sores) and pharyngitis (sore throat with redness). Occasionally, there also may be exudate (white patches on the tonsils) and usually the lymph nodes in the neck are enlarged (adenitis). Episodes of fever start suddenly and last for 3-7 days. Fevers occur routinely every few weeks; often, families know the exact day when an episode will start. Some children have other symptoms like joint pain, abdominal pain, rash, headache, vomiting or diarrhea. Children are completely well between episodes.
The disease may last for several years but usually will resolve by itself in the second decade of life. However, in nearly 15 percent of patients episodes (although less frequent) may continue to occur during adulthood. Over time, the time between the episodes will increase. Children with PFAPA continue to grow and develop normally.

It seems to be completely idiopathic, but may have some genetic component.  It pretty much a clinical diagnosis.  It usually ends in the second decade of life.  Steroid may help and tonsillectomy and adenoidectomy is often curative.


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