Table 4.

Classical diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) as defined by Thomas et al. 27

Diagnostic criteria for PFAPA

Regularly recurring fevers with an early age of onset (<5 years of age)

Symptoms in the absence of upper respiratory tract infection with at least one of the following clinical signs:

Aphthous stomatitis

Cervical lymphadenitis

Pharyngitis

Exclusion of cyclic neutropenia

Completely asymptomatic interval between episodes

Normal growth and development