Marshall Syndrome (PFAPA) – Diagnosis, Evolution, Treatment

Abstract

Marshall syndome or PFAPA (Periodic Fever, Aphthous lesions, Pharyngitis, cervical Adenitis) is a clinical entity including recurrent episodes of high fever (39- 40°C), every 4-8 weeks and lasting for 3-6 days, cervical adenitis, pharyngitis and aphthous stomatitis. PFAPA has the onset before 5 years of age. The patients have no clinical symptoms between episodes and the neurological and physical development is normal. Diagnosis of PFAPA is based on suggested clinical criteria with the exclusion of other periodic fever syndromes. Fever episodes are associated with elevated erythrocyte sedimentation rate, C reactive protein and leukocytosis. The therapeutic options are oral corticosteroids and tonsillectomy.