Abstract
PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis, Marshall's syndrome is one of the periodic febrile syndromes and is meant to be an auto-inflammatory idiopathic disease. Recurrent (periodic) febrile episodes along with aphtous stomatitis, pharyngitis or tonsillitis and cervical adenitis (lymphadenopathy) are typical clinical signs.
Another associated symptoms as vomiting, abdominal pain, diarrhea, arthritis, conjuctivitis or rash may occur. Children with initial manifestation of disease are usually younger than 5 years of age.
Between acute onsets of attack they are entirely free of any clinical symptoms. The origin of this syndrome is unknown.
The immune dysregulation of the tonsillar lymphatic tissue has been suspected. Diagnosis is difficult to confirm and is made by exclusion.
During acute episodes nonspecific inflammatory markers are often elevated in laboratory studies. Corticosteroids can have a significant effect on resolution of symptoms.
There is no causal treatment so far, surgical intervention can be considered based on assumption that adeno-tonsillectomy eliminates possible source of infection and remission of disease may occur. PFAPA syndrome causes periodic febrile episodes in early childhood relatively often.
Differential diagnosis should consider and include several recurrent fever syndromes and their causalities, based on clinical studies and laboratory investigations and detailed history as well. An early diagnosis, appropriate therapeutic management and also more timely surgical intervention (tonsillectomy) may prevent costly and unnecessary investigations and will protect the patients from irrational and frequent antibiotic treatment.