Syndrome of periodic fever, aphtous stomatitis, pharyngitis and adenopathy: results of a restrospective survey in France.

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 degrees C) and accompanying aphtous stomatitis, pharyn gitis, and cervical adenopathy (PFAPA). In 1998 a national retrospective st udy 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 betwe en the age of 3 months and 12 years, with a mean age of 5 years; no geograp hical or ethnic predisposing factors were noted. The diagnosis of symptoms was subsequently established at an age ranging from 5 months to 16 years, w ith a mean age of 6.5 years. If was determined that following an initial ph ase of generalized clinical manifestations (asthenia, cranial neuritis, dys phagia, anorexia), the symptoms become stereotyped, with the sudden appeara nce of high fever (> 40 degrees C), shivering, aphtous stomatitis, pharyngi tis, and cervical adenopathy. Other symptoms such as cranial neuritis, arth ralgia, 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 diag nostic value. The main characteristic of Marshall's syndrome is ifs periodi c 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 t o 28 days reported in the literature. After excluding the presence of an in fection, the differential diagnosis includes the following. familial Medite rranean fever, hyper IgD syndrome, and feverish neutropenia. During the per iods of fever, an inflammatory syndrome with hyperleucocytosis and a marked increase in C-reactive protein (CRP) levels and sedimentation rate is obse rved. The most effective treatment seems to be the early administration of corticoids during the initial phase, prior to the appearance of more specif ic 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 (C) 2000 Editio ns scientifiques et medicales Elsevier SAS.