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Genetic and Rare Diseases Information Center (GARD)

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Periodic fever, aphthous stomatitis, pharyngitis and adenitis


Other Names for this Disease
  • Marshall Syndrome
  • PFAPA
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Overview


Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) is a periodic disease, which is a heterogeneous group of disorders characterized by short episodes of illness that regularly recur for several years alternated with healthy periods.[1] PFAPA is characterized by high fevers lasting three to six days and recurring every 21 to 28 days, accompanied by some or all of the signs noted in its name, namely mouth sores (aphthous stomatitis), sore throat (pharyngitis), and enlarged lymph nodes (cervical adenitis). The syndrome usually occurs in children younger than five years; although it has been reported in children up to 13 years.[2] The syndrome is sporadic and non-hereditary.[3] The course of PFAPA can be persistent for years before spontaneous, full resolution.[4]
Last updated: 10/7/2013

References

  1. Berlucchi M, Nicolai P. Marshall’s syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet encyclopedia. 2004; http://www.orpha.net/data/patho/GB/uk-PFAPA.pdf. Accessed 10/7/2013.
  2. Ovetchkine P, Bry ML, Reinert P. [Marshall syndrome: results of a retrospective national survey].. Arch Pediatr. 2000; http://www.ncbi.nlm.nih.gov/pubmed/10941483. Accessed 10/7/2013.
  3. Kurtaran H, Karadað A, Catal F, Aktaþ D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr. 2004; http://www.ncbi.nlm.nih.gov/pubmed/15641271. Accessed 10/7/2013.
  4. Long S.. Tonsillectomy as treatment of PFAPA syndrome. Journal of Pediatrics. 2007;
Your Questions Answered
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Basic Information

  • The Cleveland Clinic provides an overview of the different types of periodic fever syndromes.
  • The Merck Maual provides information on periodic fever syndromes in general. The Merck Manuals are a series of healthcare books for medical professionals and consumers.
  • TheDoctorsDoctor.com web site provides additional information on PFAPA syndrome. Click on the link above to view this information page. 

In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Periodic fever, aphthous stomatitis, pharyngitis and adenitis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles