Table I.

Diagnostic criteria of PFAPA based on Marshal [5]

1. Regularly recurring fevers with early age of onset (patients < 5 years of age)

2. Constitutional symptoms in the absence of upper respiratory infection with at least 1 of the following clinical signs:

aphthous stomatitis

Pharyngitis

cervical lymphadenitis

3. Exclusion of cyclic neutropenia

4. Completely asymptomatic interval between episodes (resolve spontaneously within 4–5 days)

5. Normal growth and development without long-term sequelae of the disease