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

Alisson A. R. Fonseca, Karen Cherubini


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.


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