Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA)

PFAPA syndrome (acronym of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common cause of periodic fever in childhood. Nowadays, it is considered part of the wide family of the autoinflammatory diseases, but a genetic or molecular marker hasn't been identified yet, therefore, its etiology is still unknown. Diagnosis is essentially based on clinical criteria but, especially in younger children, it is sometimes difficult to differentiate it from other hereditary periodic fever syndromes. Fever attacks in PFAPA have a spontaneous resolution and in a high rate of patients the syndrome ends spontaneously over time. Treatment is still a matter of debate. Usually a single administration of oral corticosteroids aborts attacks. Tonsillectomy may be an alternative option but its role remains to be clarified.