PFAPA syndrome (Pediatrics - PEDS)

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PFAPA syndrome (periodic fever, aphthae, pharyngitis and cervical adenopathy) or Marshall syndrome is a cause included in recurrent or periodic fever conditions in pediatrics.

It is a chronic disease of unknown etiology. Although it probably does not have a genetic cause, it is usually grouped together with the hereditary syndromes of periodic fever. The syndrome usually begins between the ages of 2 and 5 years and tends to be more frequent among boys.

With an approximate frequency of once a month, the child has an episode of fever that lasts between 3 and 6 days. The syndrome produces fatigue, chills and, in some cases, abdominal pain and headache, as well as fever, sore throat, thrush and swollen lymph nodes. The child is healthy in between successive episodes and their growth is normal.

Diagnosis is based on medical questioning and physical examination. Blood tests are performed to measure the concentration of substances that indicate inflammation (markers).

No treatment is required, but administration of corticosteroids may relieve the child's symptoms.

Bibliographic references
  1. Shai Padeh, MD. Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome). Uptodate. Mar 29, 2017.
  2. Kastner DL, Aksentijevich I, Goldbach-Mansky R. Autoinflammatory disease reloaded: a clinical perspective. Cell 2010; 140:784.
  3. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr Med Assoc J 2008; 10:358.
  4. Sampaio IC, Rodrigo MJ, Monteiro Marques JG. Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome. Pediatr Infect Dis J 2009; 28:254.
  5. Padeh S. Periodic fever syndromes. Pediatr Clin North Am 2005; 52:577.
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Symptoms

    Localized abdominal pain


    Sore throat


    Skin rash


    Watery diarrhea


    Mouth ulcer or sore

Symptoms to watch out for

Fever not relieved by antipyretics (paracetamol, ibuprofen)

Self-care

Relative rest, rest until symptoms subside.
Take over-the-counter pain relievers or anti-inflammatories.
Consult with your primary care physician regarding the prescription of oral corticosteroids.