Marshall’s Syndrome – A Review

Marshall’s syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is a pediatric periodic disease characterized by recurrent febrile episodes associated with head and neck symptoms. The origin of this syndrome, which can last for several years, is unknown. During healthy periods, patients grow normally. Differential diagnosis includes other diseases characterized by periodic fevers such as recurrent tonsillitis, several infectious diseases, juvenile idiopathic arthritis, Behçet’s disease, cyclic neutropenia, familial Mediterranean fever, familial Hibernian fever, and hyperglobulinemia D syndrome. Many treatments have been used with various results including antibiotics, non-steroid anti-inflammatory drugs, acetylsalicylic acid, colchicine, antiviral medicines, steroids, cimetidine, and tonsillectomy. Based on our experience and analysis of the literature, surgery (tonsillectomy with or without adenoidectomy) is likely to guarantee the best results in the management of PFAPA syndrome.