Authors:

Villanueva Rodríguez C^a, Caballero Soler J^b

^aPediatra. Centro de Salud de Utebo. Zaragoza. España.
^bMIR Medicina de Familia. Hospital Clínico Universitario. Zaragoza. España.

Correspondence: C Villanueva. Email: cvillanuevar@salud.aragon.es

Reference of this article.:

Villanueva Rodríguez C, Caballero Soler J. El síndrome de Marshall o PFAPA debe ser sospechado en la consulta de Atención Primaria. Rev Pediatr Aten Primaria. 2009;11:613-7.

Published in Internet: 31/12/2009

Abstract:

Introduction: PFAPA Syndrome (periodic fever, aphtas, pharyngitis and cervical adenopathies) is one of the causes included in recurrent or periodical fever entities in pediatrics. It is characterized by repeated episodes of high fever, pharyngitis, cervical adenopathies and aphtous stomatitis. Its etiopathogeny is unknown. Diagnosis is clinical-based and laboratory findings are inespecific. A single dose (1mg/kg) of oral prednisone resolves the episode and this spectacular outcome is used as diagnostic criterion.

Case report: we report the case of a patient of 5 years diagnosed of PFAPA syndrome after some months of characteristic clinical outbreaks.

Discussion: periodic episodes of high fever, pharyngitis and cervical adenitis with poor response to conventional treatment should alert us about the PFAPA syndrome. Thinking about this entity will help to improve the diagnosis and therapeutic approach, further more reducing the parental anxiety.

Keywords: Marshall's Syndrome. PFAPA syndrome. Periodic fever.

Marshall or PFAPA syndrome should be suspected in the Primary Care setting