PFAPA and Its Association with Facial Swelling

PFAPA (periodic fever, apthous stomatitis, pharyngitis, cervical adenitis) is a rare condition affecting children with an unknown cause. Although the precise cause is unknown, inflammatory, immunological, or genetic factors have been proposed. PFAPA is a diagnosis of exclusion and requires other common causes of fever are ruled out. Although management is essentially supportive; use of corticosteroids, tonsillectomy and Cimetidine has been shown to be effective in some. Although PFAPA has been linked to abdominal pain and genital ulcers, unilateral transient facial swelling has not previously been reported. The authors present a hitherto unreported link between PFAPA and recurrent episodes of unilateral facial swelling.