Le syndrome de Marshall a été décrit pour la première fois en 1987 à propos de 12 enfants présentant une fièvre périodique associée à des aphtes buccaux, une pharyngite et des adénopathies cervicales. Une enquête rétrospective nationale initiée en France par le Groupe de pathologie infectieuse pédiatrique a permis l'analyse sémiologique de 22 cas. Ce syndrome concerne essentiellement les jeunes enfants, sans prédominance géographique ni ethnique, avec un début avant l'âge de trois ans. Après une phase de prodromes (asthénie, céphalées, dysphagie, anorexie), les épisodes sont stéréotypés, avec la survenue brutale d'une fièvre élevée (supérieure à 40 °C), des frissons, des aphtes buccaux, une pharyngite et des adénopathies cervicales. La moitié des enfants présentent des arthralgies ou des douleurs abdominales. Après avoir éliminé une infection, les principaux diagnostics différentiels sont la fièvre méditerranéenne familiale (maladie périodique), le syndrome hyper-IgD et la neutropénie fébrile. La périodicité de survenue des épisodes de fièvre varie de six à neuf semaines selon les enfants. Lor des épisodes fébriles il existe un syndrome inflammatoire avec hyperleucocytose et élévation franche de la protéine C-réactive et de la vitesse de sédimentation. Le traitement le plus efficace semble être l'administration précoce de corticoïdes dès les prodromes. Le pronostic est excellent, avec un espacement progressif des crises, et l'absence de complication. L'étiologie demeure inconnue.

Marshall's syndrome or periodic fever syndrome was first described in 1987 in the USA based on observations of 12 children under the age of five with periodic fever (> 38 °C) and accompanying aphtous stomatitis, pharyngitis, and cervical adenopathy (PFAPA). In 1998, a national retrospective study was carried out in France by the pediatric infectious pathology group, and a semeiological analysis was made of 22 cases. The main characteristics of Marshall's syndrome found in this patient population were in agreement with those reported in the literature. The onset of symptoms occurred between the age of 3 months and 12 years, with a mean age of 5 years; no geographical or ethnic predisposing factors were noted. The diagnosis of symptoms was subsequently established at an age ranging from 5 months to 16 years, with a mean age of 6.5 years. It was determined that following an initial phase of generalized clinical manifestations (asthenia, cranial neuritis, dysphagia, anorexia), the symptoms become stereotyped, with the sudden appearance of high fever (> 40 °C), shivering, aphtous stomatitis, pharyngitis, and cervical adenopathy. Other symptoms such as cranial neuritis, arthralgia, and abdominal pain may also be present (50% of cases in the present study), but due to their variability of appearance they are of lesser diagnostic value. The main characteristic of Marshall's syndrome is its periodic aspect; with fever occurring every 6 to 9 weeks, with a mean interval of 66 days before recurrence of fever compared to the shorter interval of 21 to 28 days reported in the literature. After excluding the presence of an infection, the differential diagnosis includes the following: familial Mediterranean fever, hyper IgD syndrome, and feverish neutropenia. During the periods of fever, an inflammatory syndrome with hyperleucocytosis and a marked increase in C-reactive protein (CRP) levels and sedimentation rate is observed. The most effective treatment seems to be the early administration of corticoids during the initial phase, prior to the appearance of more specific symptoms. The prognosis is excellent, with a progressive decrease in the incidence of periodic fever and an absence of complications. However, the etiology of Marshall's syndrome has not yet been determined.