Clinical aspects of PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and adenitis) <b>[Abstract in English]</b>

Abstract

Purpose: The aim of this work was to present a literature review about PFAPA syndrome, focusing its clinical features, diagnosis and treatment.
Data source: Articles indexed on MEDLINE published from 1984 to 2003 were reviewed.
Data synthesis: PFAPA syndrome is a benign disease characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis and cervical adenitis affecting children under age of five years. Children affected are healthy between the episodes, which recur every 28 days. Early administration of prednisone is effective in controlling symptoms, although patients can show spontaneous remission of clinical manifestations as they become older. There are no long-term sequelae.
Conclusion: Correct diagnosis of PFAPA is important, especially to avoid misdiagnosis with Behçet syndrome, hypergammaglobulinemia D syndrome, familial Mediterranean fever and cyclic neutropenia. As confirmed PFAPA diagnosis, we have to explain parents about the benign character of this disease as well the possibility of spontaneous remission of its manifestations at puberty.
KEY WORDS: RELAPSING FEVER; PHARYNGITIS; STOMATITIS, APHTHOUS; LYMPHADENITIS; CHILD.