PFAPA with facial swelling- a new association?

PFAPA (periodic fever, apthous stomatitis, pharyngitis, cervical adenitis) is a rare condition of unknown cause affecting children. Although the exact etiology is unknown, inflammatory, immunological or genetic causes have been suggested. The diagnosis is made by exclusion of other causes of periodic fever. Although management is essentially symptomatic, single corticosteroid dose, tonsillectomy and Cimetidine has been shown to be associated with resolution of symptoms. Although abdominal pain and genital ulcers have been reported in association with PFAPA, unilateral transient facial swelling has not been previously reported. The authors present a hitherto unreported association of PFAPA with recurrent episodes of unilateral facial swelling.