Marshall syndrome – a challenge in medical practice

Abstract

Marshall syndrome (periodic fever, adenitis, pharyngitis, aphthae, PFAPA syndrome) is characterized by recurrent episodes of fever associated with aphthous stomatitis, cervical adenitis or pharyngitis. Although it is the most common cause of recurrent fever in children, the diagnosis is rarely established. The aim of this study was to describe a group of Romanian children with Marshall syndrome. In seven children with PFAPA, the following methods were used: patient history, clinical examination, and determination of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and tumor necrosis factor α (TNF-α). The treatment consisted in prednisone at a dose of 1 mg/kg. The age at onset was 2.2 years and at diagnosis 4.8 years. The mean interval between episodes was 3.1 weeks and the duration per febrile episode was 3.7 days. The patients presented with pharyngitis (100%), adenitis (100%) and aphthous lesions (57.1%). The mean ESR value was 31 mm/h, CRP 7.8 mg/dL and leukocytes 17700/mm3. TNF-α remained elevated between febrile episodes. Six patients treated with prednisone had favorable evolution. In conclusion, PFAPA should be suspected in children with periodic fever associated with pharyngitis, cervical adenitis and aphthous stomatitis.